Skip to main content

Full text of "Lectures on the diseases of the nervous system, delivered at La Salpêtrière"

See other formats 


Wi 

i 

;i,j,'.i,-T  i;  f'/ifi','' 


:r'^'  1- 


■ .-    •  r    '  >    *  I    'I    •  r    *\ 

f,  Ji  1.  'i  >i  'i  'r  ' 

■l  i.-'l  'l  li  •(  'l  • 
«1  'l  fl''l  'l  *■  '■ 


'i.'«,'r. '••'»•'•. '«-'i.'! 


X 


<v 


?L^  J 


-l'en    >.  h. 


-''.  -Sy 


THE    NEW    SYDENHA 

SOCIETY. 


INSTITUTED    MDCCCLVIII. 


VOLUME   XC. 


/ 

^ 


LECTUEES 


THE   DISEASES   OF 

THE  NEMOUS   SYSTEM. 

DELIYERED    AT    LA    SALPETRIERE 


J.    M.    GHAEGOT,   %2'^^\^lil 

PKOÎESSOR  IN  THE  ÏACULTÏ   OF  MEDICTNE  Oï  PABIS  ;  PHYSICIAN  TO  LA  SALPÊTBIÈBE  ;  MEMBEE 

OE   THE   ACADEMY   OF   MKDIC1NE,  OF   THE    CLINICAL   SOCIETY    OF   LONDON,  OF  THE 

CLINICAL    SOCIETY    OF   BUDA-PESTH,  OF    THE   MEDICAL   SOCIETY  OF   NATUEAL 

SCIENCES    OF   BEVSSELS;   PEESIDENT  OF   THE    ANATOMICAL   SOCIETY,  AND 

EX-VICE-PBESIDENT   OF   THF,  BIOLOGICAL   SOCIETY    OF   PABIS,   ETC. 


SECOND    SEEIES. 


TRANSLATED  AND  EDITED  BY 

GEORGE   SIGEESON,   M.D.,  Ch.M., 

LICENTIATE    OF  THE    KING  AND    QUEEN'S    COLLEGE  OF   PHYSICIANS  OF  lEELAND  ;     DEAN    OF    THE 

FACULTY    OF    SCIENCE,    C.U.I.  ,    MEMBEE   OF    THE    EOYAL   IBISH   ACADEMY;    FELLOW  OF 

THE   HNNEAN    SOCIETY    OF   LONDOM;    MKMBKR   OF    COUKCIL   OF   STATISTICAL 

SOCIETY,  DUBLIN  ;     MEMBEE   OF   THE    SCIENTIFIC   SOCIETY   OF   BEUSSELS  ; 

COEEESPONDING  MEMBEE  OF   THE    CLINICAL   AND    ANTHEOPOLOGICAL 

SOCIETIES   OF  PAEIS  ;    ETC. 


THE     NEW     SYDENHAM     SOCIETY, 
LONDON. 

MDCOCLXXXI. 


(LL 


LONDOH 
PEINXED  BY  J.   E.   ADLAED,  BAETHOLOMEW   CLOSE. 


NOTICE 


AccoMPANYiNG  tliis  volume  o£  Professer  Charcot's 
'^  Lectures  on  the  Diseases  of  the  Nervous  System,'  the 
reader  will  find  a  number  of  plates  and  diagrams,  with 
explanatory  descriptions.  Thèse  serve  to  illustrate 
not  only  tliis,  but  also  the  first  séries  of  Lectures 
ah^eady  published  by  '  the  New  Sydenham  Society.' 
Références  to  thèse  illustrations  may  be  observed 
in  the  text  of  the  translation  of  both  volumes. 

G.  S. 


CONTENTS. 


LECTURE  I. 

ANATOMICAL  INTRODUCTION. 

PAGE 

SuMMAKY. — The  task  of  the  clinical  observer  differs  from  that  of  the 
nosographer.  Diversity  of  opinions  concerning  the  seat  of  lésion  in 
locomotor  ataxia.  Insufiiciency  of  the  methods  of  research  employed. 
Sclerosis  of  the  posterior  columns  :  its  significance.  Appearances 
presented  on  examination  by  the  naked  eye.  First  cases  of  sclerosis 
of  the  posterior  columns. 

Macroscopical  examination. — Grey  induration  ;  its  characters.  Original 
focus  of  the  irritative  lésion. 

Microscopical  examination. — Topographical  anatomy.  Spinal  circum- 
scribed  régions.  Goll's  column.  External  bands  of  posterior 
fasciculi  ;  their  altération  seems  constant  in  locomotor  ataxia. 

Systematic  médian  fasciculated  sclerosis.  Latéral  fasciculated  sclerosis. 
In  locomotor  ataxia,  sclerosis  makes  its  first  appearance  in  the 
postero-lateral  bands.  Relationship  between  lésions  and  symptoms. 
Manner  in  which  sclerosis  is  propagated.  Internai  radicular  fasciculi. 
Invasion  of  the  anterior  grey  cornua  ;  consécutive  amyotrophy.        .        3 


LECTURE  IL 

RETROGRADE  ACTION  IN  SPINAL  DISEASES;  ITS  RELATION 
WITH  SCLEROSIS  OF  THE  POSTERIOR  COLUMNS;  LIGHT- 
NING  PAINS  AND  GASTRIC  CRISES. 

St:mm:ab,y. — Relations  between  the  internai  radicular  nerve-filaments  and 
the  groups  of  multipolar  nerve-cells  of  the  anterior  cornua.  Récurrent 
or  rétrograde  symptoms.  Sclerosis  of  the  posterior  médian  bundles  ; 
propagation  of  the  sclerosis  to  the  latéral  bands  of  the  posterior 
columns. 


Viii  CONTENTS. 

PAGE 

Classical  type  of  progressive  locomotor  ataxia.  Prodromic  period  of 
shootiug  paius.  Period  of  established  disease,  or  of  tabetic  incoordi- 
nation.    Paralytic  period. 

Liglitning  pains.  Their  varieties;  boring  pains;  lancinating  pains; 
constrictive  pains.  Symptomatic  sliooting  pains  in  disseminated 
sclerosis,  progressive  gênerai  paralysis  and  chronic  alcoholism. 
Viscéral  symptoms  ;  vesical,  uretliral  pains  ;  rectal  tenesraus. 

Gastric  crises  ;  spécifie  characters.  Duration.  Abortive  cases  of  loco- 
motor ataxia  ......  .18 


LECTURE  III. 

TABETIC  AMAUROSIS. 

SuMMABY. — Cephalic  symptoms  in  locomotor  ataxia.  Lésions  of  tlie 
cranial  and  bulbar  nerves.  Progressive  grey  induration  of  tlie  optic 
nerve.  >'  Progressive  atrophy  of  the  papilla.  Necessity  of  ophthal- 
moscopic  examination  in  the  diagnosis  of  some  cérébral  affections. 

Isolated  existence  of  tabetic  amaurosis  ;  its  frequency.  Characters  of 
the  anatomic  lésion  of  the  optic  nerve,  seen  by  the  naked  eye  and 
uuder  the  microscope.  Appearance  of  papilla;  1°,  in  the  normal 
state  ;  2°,  in  prog-ressive  grey  induration.  Functional  dérangements 
accompanying  grey  induration  of  the  optic  nerve  and  papilla.  Modi- 
fication of  the  papilla  in  cases  of  embolism  of  the  arteria  centralis  of 
the  retina,  in  glycosuria,  syphilitic  retino-choroiditis,  and  gênerai 
paralysis. 

Neuro-retinitis  ;  its  forms  and  symptoms  ;  différences  which  distinguish 
it  frora  tabetic  amaurosis.  Clinical  facts  demonstrating  the  im- 
portance of  the  signs  furnished  by  the  ophthalmoscope  .  .    34 

LECTUEE  IV. 

ON  SOME  VISCERAL  DERANGEMENTS  IN  LOCOMOTOR  ATAXIA. 
ARTHROPATHIES  OE  ATAXIC  PATIENTS. 

SuMMAEY.— Disorders  of  the  genito-urinary  organs.  Fréquent  désire  to 
micturate  ;  satyriasis  ;  rectal  tenesmus.  Oculo-pupillary  symptoms. 
Permanent  accélération  and  dicrotism  of  the  puise. 

Arthropathy  of  ataxic  patients;  its  frequency.  Cases.  This  arthro- 
pathy  is  developed  at  a  but  slightly  advanced  period  of  the  spinal 
disease.  Prodromes.  Phases  of  ataxic  arthropathy.  Joints  attacked. 
Its  spécial  characters.  Diagnosis  between  the  arthropathy  of  ataxic 
patients  and  dry  arthritis. 

Anatomical  lésions.  Arthropathies  consécutive  on  affections  of  spinal 
origin.  Mechanisra  of  production  of  ataxic  arthropathies.  Lésions 
of  the  anterior  cornua  of  the  grey  substance.    Desideratum       .       .     47 


CONTENTS.  IX 


LECTURE  V. 

ON    SLOW    COMPRESSION    OF    SPINAL    CORD.    ANATOMICAL 

PREFACE. 

PAGE 

SuMMARY. — Slow  compression  of  cord  due  to  various  causes  ;  importance 

of  its  study. 
Causes  of  compression.    Tumours  of  tlie  cord  ;  glioma,  tubercle,  sarcoma, 

carcinoma,  gumma,  kystic  dilatation  of  tlie  cord. 
Tumours  primarily  developed  in  the  méninges.    Sarcoma,  psammoma, 

echinococci,  inflammatory  neoplasias  (internai  pachymeningitis,  hyper- 
trophie pacbymeningitis). 
Morbid  productions  formed  in  the  cellulo-adipose  tissue  of  the  rachis. 

Carcinoma,  sarcoma,  hydatic  kysts,  abscess. 
Vertébral  lésions.     Syphilitic  hyperostoses,  dry  arthritis,  Pott's  disease. 

Meehanism  of  compression  of  the  cord.     Cancerous  vertébral  disease. 

Painful  paraplegia  of  cancerous  patients  .  .  .  .65 


LECTURE   VI. 

ON  SLOW  COMPRESSION  OF  THE  SPINAL  CORD.  ANATOMICAL 
MODIFICATIONS  IN  CASES  WHICH  TERMINATE  IN  CURE. 
SÏMPTOMS.  PSEUDO-NEURALGIAS.  PAINFUL  PARAPLEGIA 
OF  CANCEROUS  PATIENTS. 

SuMMAKY, — Anatomical  modifications  which  the  cord  undergoes  at  the 
compressed  point.  Changes  in  form  :  softeniug,  induration.  Inter- 
stitial  myelitis.  Ascending  and  descending  consécutive  sclerosis. 
There  may  be  restoration  of  functiou,  notwithstanding  the  existence 
of  profound  lésions.  Degeneration  of  nerve-tubes  at  the  compressed 
point. 

Symptoms.  Extrinsic  and  intrinsic  symptoms.  Topographical  ana- 
tomy  of  the  vertébral  région. 

Extrinsic  symptoms  ;  pseudo-neuralgias.  Pseudo-neuralgias  in  cases  of 
rachidian  tumours,  of  Pott's  disease,  and  in  cancerous  vertébral 
disease. 

Painful  paraplegia  of  cancerous  patients .  Pain,  its  characters,  paroxysms . 
Tegumentary  hypersesthesia.  Eruption  of  zona  along  the  course  of 
painful  nerves;  circumscribed  cutaneous  anœsthesia;  partial  muscular 
atrophy  and  contracture.  Déformation  of  the  vertébral  column, 
Difficulty  of  diagnosis  in  certain  cases  :  osteomalacia,  hypertrophie 
cervical  pachymeningitis,  spinal  irritation,  &c.  .  .  «7^ 


CONTENTS. 


LECTUEE  VII. 

ON  SLOW  COMPRESSION  OF  THE  SPINAL  CORD.  SYMPTOMS. 
DISORDERS  or  MOÏILITY  AND  OF  SENSIBILITV  CONNECTED 
WITH  SPINAL  COMPRESSION.  SPINAL  HEMIPLEGIA  AND 
HEMIPARAPLEGIA,  WITH  CROSSED  AN^STHESIA. 

PAGE 

SuMMARY. — Lésions  of  the  cord  at  the  point  of  compression.  Tiiey 
occupy  the  whole  extent  of  the  cord,  in  a  transverse  direction,  or 
only  affect  one  of  its  latéral  halves. 

First  case  :  Succession  of  symptoms.  Prédominance  of  motor  dis- 
orders  at  the  outset  ;  paresis,  paralysis  with  flaccidity,  temporary 
rigidity,  permanent  contracture  of  limbs,  augmentation  of  reflex  ex- 
citability.  Disorders  of  micturition  ;  Budge's  theory.  Modification 
of  seusibility  ;  delay  in  the  transmission  of  sensations  ;  dyssesthesia. 
Associated  sensations. 

Second  case  ;  Lésions  bearing  on  one  of  the  latéral  halves  of  the  spinal 
cord.  Lésion  circumscribed.  Spinal  hemiparaplegia  with  crossed 
ansethesia  ;  its  characters.     Spinal  hemiplegia  .  .  -93 


LECTUEE  YIII. 

ON  SLOW  COMPRESSION  OF  THE  SPINAL  CORD.  CERVICAL 
PARAPLEGIA,  PECULIAR  SYMPTOMS.  PERMANENT  SLOW 
PULSE. 

SuMMARY. — On  cervical  paraplegia.  Compression  of  nerves  of  upper  ex- 
tremities.  Lésions  of  spinal  cord  in  the  neck  ;  their  mode  of  action 
in  the  production  of  cervical  paraplegia.  Distinction  between  cervical 
paraplegia  due  to  the  compression  of  peripheral  nerves  and  that 
dépendent  on  a  lésion  of  the  cord.  Altération  of  motor  nerve-cells  and 
tumours  of  the  cord — causes  of  a  third  form  of  cervical  paraplegia. 

On  some  peculiar  symptoms  of  slow  compression  of  the  cervical  cord. 
Oculo-pupillary  disorders.  Cough  and  dyspnœa.  Frequently  re- 
curring  vomiting.  Difficulty  of  déglutition.  Hiccough.  Functional 
dérangement  of  bladder.     Epiieptic  attacks. 

On  permanent  slow  puise.  Temporary  slowing  of  puise  in  fractures  of 
vertebrse  of  neck.  Permanent  slow  puise  connected  with  certain 
organic  affections  of  heart  (aortic  insufficiency,  fatty  degeneration  of 
cardiac  tissue,  fibrinous  deposits).  InsufSciency  of  cardiac  lésions,  in 
some  cases,  to  explain  slow  puise.  Phenomeua  connected  with 
permanent  slow  puise.  Syncope,  apoplectiform  state,  convulsive  fits. 
In  certain  cases,  the  starting  point  of  permanent  slow  puise  to  be 
sousrht  in  cervical  cord  or  bulbus  rachidicus.    Dr.  Halberton's  case. 


CONTENTS.  XI 

PASE 

Sudden  deatli  from  rupture  of  transverse  ligament  of  tbe  odontoid 

process. 
Symptoms  accompauying  lésions  of  the  lumbar  enlargement  and  cauda 

equina  ........  io8 


LECTUEE  IX. 

INFANTILE  PARALYSIS. 

SuMMARY. — Spinal  myopathies  or  myopathies  of  spinal  origin.  General 
characters.  Localisation  of  spinal  lésions  in  the  anterior  cornua  of 
grey  matter. 
Infantile  spinal  paralysis.  To  be  considered  as  a  disease  for  study. 
Symptoms.  Period  of  invasion,  its  modes  ;  second  period  or  retro- 
gression  of  symptoms  with  localisation  of  muscular  lésions  (muscular 
atrophy,  arrest  of  development  of  the  osseous  system,  coldness  of 
extremities,  deformities,  paralytic  club-foot). 
Pathological  anatomy  of  infantile  paralysis.  Lésions  of  muscles  at 
différent  periods;  fatty  deposits.  Lésions  of  the  nervous  system; 
history  (Charcot  and  Cornil,  Vulpian  and  Prévost,  Charcot  and 
Joffroy,  Parrot,  Lockhart  Clarke  and  Johnson,  Damaschino  and 
Roger).  Localisation  of  lésions  in  the  anterior  cornua  of  grey  sub- 
stance. Secondary  altérations  ;  scierons  transformation  of  neuroglia  ; 
foci  of  disintegration  ;  partial  sclerosis  of  the  aiitero-lateral  columns  ; 
atrophy  of  the  anterior  roots.  Reasons  tending  to  demonstrate  that 
the  primary  lésion  résides  in  the  nerve-cells  .  .  -125 


LECTUEE  X. 

SPINAL  PARALYSIS  OP  THE  ADULT.  NEW  RESEARCHES  CON- 
CERNING  THE  PATHOLOGICAL  ANATOMY  OF  INFANTILE 
SPINAL  PARALYSIS.  AMYOTROPHIES  CONSECUTIVE  ON 
ACUTE  DIFFUSE  SPINAL  LESIONS. 

SuMMAKY. — Spinal  paralysis  of  the  adult.  History.  Description  of  a 
case,  borrowed  from  M.  Duchenne  (de  Boulogne).    Personal  facts. 

Close  analogies  Connecting  acute  spinal  paralysis  of  the  adult  with 
that  of  the  child.  Symptomatological  modifications  due  to  âge. 
Prognosis. 

Récent  works  concerning  the  pathological  anatomy  and  physiology  of 
infantile  spinal  paralysis  ;  they  coufirm  in  essential  points,  and  com- 
plète in  certain  respects,  the  results  already  detailed, 

A  word  as  to  acute  spinal  lésions  which  are  net,  as  in  infantile  paralysis. 


-Xll  CONTENTS. 


systematically  limited  to  tbe  auterior  cornua  of  the  grey  substance. 
Acute  central  generalised  myelitis,  hœmatomyelia,  traumatic  myelitis, 
acute  partial  myelitis.  Conditions  in  which  thèse  affections  détermine 
the  rapid  atrophy  of  the  muscles  .  .  .  .  .144 


LECTURE  XL 

CHRONIC  SPINAL  AMYOTROPHIES.  PROTOPATHIC  SPINAL 
PROGRESSIVE  MUSCULAR  ATROPHY  (DUCHENNE-ARAN 
TYPE). 

SuMMAUY. — Clinical  varieties  of  cases  designated  under  the  name  of  pro- 
gressive muscular  atrophy  (spinal  progressive  muscular  atrophies). 
Uniformity,  in  thèse  cases,  of  the  spinal  lésion  which  affects  the 
anterior  cornua  of  the  grey  substance. 

Study  of  protopathic  spinal  progressive  muscular  atrophy  as  typical  of 
the  group  :  simple  spinal  lésion.  Chronic  deuteropathic  spinal 
amyotrophies.  The  lésion  of  motor  nerve-cells  is  hère  consécutive  ; 
it  is  superadded  to  a  spinal  lésion  of  variable  position.  Account  of 
the  principal  spinal  affections  which  may  produce  deuteropathic  pro- 
gressive amyotrophy;  hypertrophie  spinal  pachymeningitis  ;  sclerosis 
of  posterior  columns  ;  chronic  central  myelitis  ;  hydromyelia  ;  intra- 
spinal  tumours  ;  disseminated  sclerosis  ;  symmetrical  latéral  sclerosis. 

On  protopathic  spinal  progressive  muscular  atrophy  in  particular 
(Duchenne-Aran  type).  Symptoms  ;  individual  atrophy  of  the  muscles, 
functional  disorders  ;  prolonged  persistence  of  faradaic  contractility  ; 
fibrillary  tremors;  paralytic  déformations,  or  déviations,  "griffes." 
Modes  of  invasion.  Etiology  :  hereditary  transmission,  cold,  traumatic 
injuries. 

Pathological  anatomy.  Lésions  of  the  cord;  altération  limited  to  the 
anterior  cornua  of  the  grey  substance  (nerve-cells,  neuroglia). 
Lésions  of  the  nerve-roots  and  of  the  peripheral  nerves.  Muscular 
lésions  and  their  nature  .  .  .  .  -163 


LECTURE  Xll. 

DEUTEROPATHIC  SPINAL  AMYOTROPHIES.    LATERAL  AMYO- 
TROPHIC   SCLEROSIS. 

SuMMAUY. — Deuteropathic  spinal  amyotrophies.  Latéral  amyotrophic 
sclerosis  ;  localisation  of  the  spinal  lésion  in  the  latéral  columns. 
Causes  of  this  localisation,  elucidated  by  a  study  of  the  development 
of  tlie  spinal  cord.  Pormatiou  of  latéral  columns  ;  of  GoU's  columns 
and  of  Tûrck's  columns. 


CONTENTS. 


Latéral  sclerosis  consécutive  on  cérébral  lésion. 

Primary  symmetrical  latéral  sclerosis.  Patliological  anatomy.  Appear- 
ance  and  topography  of  the  lésion  in  the  cord  and  bulbus.  Consé- 
cutive lésions  of  the  grey  substance  (motor  uerve-cells,  neuroglia),  in 
the  cord  and  in  the  bulbus.  Secondary  changes  :  anterior  nerve- 
roots.     Peripheral  nerves.    Trophic  lésions  of  the  muscles        .        .  i8o 


LECTURE  XIII. 
ON  AMYOTROPHIC  LATERAL  SCLEROSIS.    SYMPTOMATOLOGY. 

SuMMAKY. — Facts  which  form  the  basis  of  the  symptoraatology  of  amyo- 
trophic  latéral  sclerosis.  Personal  observations.  Corroborative 
cases. 

Différences  which  clinically  separate  amyotrophic  latéral  sclerosis  from 
protopathic  spinal  muscular  atrophy. 

Symptoms  common  to  both  affections.  Progressive  amyotrophj, 
fibrillary  contractions,  préservation  of  electric  contractility. 

Symptoms  peculiar  to  amyotrophic  latéral  sclerosis.  Prédominance  of 
motor  paralysis.  Permanent  spasmodic  contracture.  Absence  of 
sensory  disorders.  Paralytic  déformations;  attitude  of  hand. 
Tremulation  of  upper  extremities  in  purposed  movements.  Modes 
of  invasion.  Cervical  paraplegia.  Invasion  of  inferior  extremities. 
Characteristics  of  the  contracture.  Bulbar  phenomena  ;  difficulty  of 
déglutition.  Impeded  speech.  Paralysis  of  the  vélum  palati,  of  the 
orbicularis  oris,  &c.     Grave  disorders  of  respiration. 

Summary  of  symptoms.    Prognosis.    Pathological  physiology  .  192 


LECTUEE  XIV. 

DEUTEROPATHIC  AMYOTROPHIES  OF  SPINAL  ORIGIN  {CON- 
CLUSION).  HYPERTROPHIC  CERVICAL  PACHYMENINGITIS, 
ETC.,  ETC. 

Summary. — Amyotrophy  connected  with  descending  latéral  sclerosis  con- 
sécutive on  a  circumscribed  lésion  of  the  brain  and  spinal  cord. 
Ulustrative  cases. 
Hypertrophie  cervical  raeningitis.  Pathological  anatomy  :  altération  of 
the  méninges  ;  of  the  spinal  cord  ;  of  the  peripheral  nerves. 
Symptoms;  painful  period  (cervical  pains,  rigidity  of  neck;  formi- 
cation  and  numbness  ;  paresis  ;  cutaneous  éruptions)  ;  second  period 
(paralysis,  atrophy,  hand-deformity  ("griffe"),  contracture,  patches 
of  ansesthesia;  paralysis  and  contracture  of  the  lower  extremities). 


XIV  CONTENTS. 


Characters  which  distiuguish  hypertrophie  cervical  pachymeningitis 

from  amyotrophic  latéral  sclcrosis. 
Amyotrophies   consécutive  on  locomotor  ataxia.      Peculiar   form   of 

muscular  atrophy  in  such  cases.     Pathogeny. 
Amyotrophy  consécutive  on  sclerosis  disseminated  in  patches. 
Subacute  spinal  gênerai  paralysis.    Analogies  with  infantile  paralysis. 

Desideratum. 
Amyotrophies  independent  of  a  lésion  of  the  spinal  cord,  examples  ; 

pseudo-hypertrophie  paralysis  ;  saturnine  amyotrophy. 
New  considérations  relative  to  the  topographie  pathological  anatomy  of 

the  spinal  cord  .......  aqg 


LECTURE  XY. 

ON  SPASMODIC  TABES  DORSALIS. 

SuMMAKY. — Provisional  dénomination  ;  its  justification  :  symmetrical  and 
primary  sclerosis  of  the  latéral  columns.  Spasmodic  tabès  dorsalis, 
and  ataxic  tabès  dorsalis.  Parallel  between  the  two  affections. 
Characteristics  of  gait. 
On  contracture  and  trépidation  in  spasmodic  tabès  dorsalis.  Absence 
of  sensory  disorders.  Invasion.  Evolution.  Mode  of  invasion  in 
the  limbs.  Prognosis  and  treatment.  Diagnosis  :  disseminated 
sclerosis  (spinal  form),  hysterical  contracture,  transverse  myelitis, 
latéral  amyotrophic  sclerosis,  &c  .  ...  233 


LECTUEE  XYI. 

URINARY  PARAPLEGIAS. 

SuAiMARY. — Preamble.  Theoretical  point  of  view.  Clinical  reality  of 
urinary  paraplegias.  DeQnitiou.  Classification  of  cases  into  three 
groups. 
Myelitis  consécutive  on  diseases  of  the  uriuary  passages.  Rare  in 
wornen  ;  fréquent  in  men.  Conditions  of  development  :  gonorrhœa, 
stricture  of  the  urethra,  cystitis,  nephritisj  prostatic  affections; 
calculous  pyelo-nephritis.  Exacerbation  of  the  disease  of  the  urinary 
passages  précèdes  the  invasion  of  spinal  phenomena.  Symptoms  : 
formication  ;  anœsthesia,  dorso-lumbar  and  girdling  pains.  Paraplegia 
with  flaccidness  ;  excitation,  then  abolition  of  reflex  excitability  ; 
permanent  contracture  ;  bedsores.  Position  and  nature  of  the  lésions. 
Pathogeny.  Propagation  of  the  rénal  lésion  to  the  cord  by  meaus  of 
the  nerves  (Troja,  Leyden).  Corroborative  experiments.  Examples 
of  propagation  of  nerve-inflammations  to  the  cord. 


CONTENTS.  XV 

PAGE 

Eefles  uriuary  paraplegias.  Symptoms.  M.  Brown-Séquard's  ex- 
planation.  Récent  experiments.  Inliibitoiy  phenomenon.  Irritation 
of  the  périphérie  nerves,  False  paraplegia.  Descending  neuritis. 
Affections  of  intestines  and  utérus  ....  249 


LECTURES  XVII  AND  XVIII. 

MÉNIÈRE'S  VERTIGO  =  VERTIGO  AB  AURE  L^SA. 

SuMMAur. — A  case  of  Ménière's  vertigo.  Description.  Habituai  vertige 
increased  by  motion.  Its  characters  :  paroxysmal  exacerbations  ; 
subjective  translation  movements.  Old  lésions  of  the  ears  ;  outflow 
of  pus,  altération  of  tympan.  Walking  and  standing  impossible. 
Evolution  of  the  disease.     Complication  :  hysterical  fits. 

Historical  sketch.  Vertigo  of  Ménière  as  yet  little  known.  Diagnosis  ; 
apoplectiform  cérébral  congestion  ;  epileptic  petit  mal  ;  gastric  vertigo. 
Relation  between  the  sudden  development  of  noises  in  the  ears  and 
the  invasion  of  sensations  of  giddiness. 

Diseases  of  the  car  :  labyrinthic  otitis  ;  médian  otitis,  catarrh,  &c. 
Prognosis.     Cure  by  deafness,    Treatment. 

Reputedly  incurable  diseases.  Examples  of  cure.  Case  of  Ménière's 
vertigo.  Situation  of  the  patient  in  May,  1875  ;  permanent  sensations 
of  giddiness;  crises  announced  by  shrill  whistling.  Motor  halluci- 
nation. Treatment  by  sulphate  of  quinine  :  doses,  effects,  remarkable 
improvement.  Another  example  of  amélioration  due  to  the  prolongea 
use  of  sulphate  of  quinine  .....  261 


LECTUKE  XIX. 
ON  POST-HEMIPLEGIC  HEMICHOREA. 

SuMMABY. — Post-hemiplegic  hemichorea.  Clinical  facts.  Disorders  of 
gênerai  and  spécial  sensibility.  Motor  disorders  :  their  resemblance 
to  choreic  movements.  Trépidation  of  hémiplégie  patients.  In- 
stability  of  members,  affected  by  post-hemiplegic  hemichorea.  Cha- 
racters of  muscular  disorders  in  rest  and  in  motion. 

Organic  lésions.  Poci  of  intra-encephalic  hgemorrhage  and  ramollisse- 
ment : — partial  atrophy  of  the  brain. 

Rarity  of  post-hemiplegic  hemichorea.  Relation  between  hemichorea 
and  hemianœsthesia.  Seat  of  lésions  to  which  thèse  symptoms 
belong  ;  posterior  extremity  of  the  optic  thalami  ;  posterior  portion  of 
the  caudate  nucleus  ;  posterior  portion  of  the  corona  radiata. 

Pr£e-hemiplegic  hemichorea  .  .  .  .  -275 


XVI  CONTENTS. 

LECTUEE  XX. 
ON  PARTIAL  EPILEPSY  OP  SYPHILITIC  ORIGIN 


SuMMARY. — Partial  or  hémiplégie  epilepsy.  Its  relations  with  cérébral 
syphilis.  Historical  considérations.  Description  of  a  case  of  partial 
epilepsy  of  syphilitic  origin.  Characters  and  peculiar  seat  of  the 
cephalalgia.     Necessity  of  energetic  therapeutical  intervention. 

Modes  of  invasion  of  the  convulsive  symptoms.  New  corroborative 
examples.  Succession  of  fits.  Appearance  of  permanent  con- 
tractures. Relations  between  the  cephalalgia  and  the  motor  région 
of  the  brain. 

Lésions.  Gummatous  pachymeningitis.  Probable  seat  of  those 
lésions. 

Mixed  treatment,  with  interruptions  ....  286 


APPENDIX. 


L — Multiple  Pathological  Luxations  and   Spontaneous  Fractures  in  a 
Patient  suffering  from  Locomotor  Ataxia.     By  J.  M.  Charcot 

IL — On  the  Tuméfaction  of  the  Motor  Nerve-cells  and  of  the  Axis 
cylinders  of  the  Nerve-tubes  in  Certain  Cases  of  Myelitis.  By  J.  M 
Charcot  ...... 

III.— Note  on  a  Case  of    Spinal   Protopathic    Progressive    Muscular 
Atrophy  (Duchenne-Aran  Type).     By  J.  M.  Charcot 

IV. — Two  Cases  of  Latéral  Symmetrical  Amyotrophic  Sclerosis 

V. — Note  on  a  Case  of  Glosso-laryngeal  Paralysis,  followed  by  Autopsy 
By  J.  M.  Charcot  ..... 

VI. — Note  upon  the  Anatomical  State  of  the  Muscles  and  Spinal  Cord  in 

a  Case  of  Pseudo-Hypertrophie  Paralysis.     By  J.  M.  Charcot 
VIL— On  Athetosis         ...... 

INDEX       

Explanation  of  Plates,  Vol.  I. 
Explanation  of  Plates,  Vol.  IL 


305 


317 

327 
341 

363 

378 
390 

395 


PART  FIBST. 


AKOMALIES   OF   LOCOMOTOR   ATAXIA. 


VOL.    II. 


LECTURE  1. 

ANATOMICAL  INTRODUCTION. 

SuMMARY. — The  tash  of  tJie  clinical  observer  differs  from  that  oj 
the  nosograpJier.  Diversity  of  opinions  concerning  ilie  seat  of 
lésion  in  locomotor  ataxia.  Insufficiency  of  the  methods  of 
research  employed.  Sclerosis  of  the  posterior  columns  :  its 
significance.  Appearances  presented  on  examination  Ity  the 
naked  eye.     First  cases  of  sclerosis  of  the  posterior  columns. 

Macroscopical  examination. —  Grey  induration:  its  charac- 
ters.     Original  focus  of  the  irritative  lésion. 

Microscopical  examination. — Topographical  anatomy.  Spinal 
cïrcumscrïbed  régions.  GolVs  column.  External  hands  of 
posterior  fasciculi  :  their  altération  seems  constant  in  loco- 
motor ataxia. 

Systematic  médian  fasciculated  sclerosis.  Latéral  fasci- 
culated  sclerosis.  In  locomotor  ataxia,  sclerosis  maîces  itsfirst 
appearance  in  the  poste7'o- latéral  hands.  Relationship  hetween 
lésions  and  symptoms.  Manner  in  which  sclerosis  is  propa- 
gated.  Internai  radicular  fasciculi.  Invasion  of  the 
anterior  grey  cornua  :  consécutive  amyotrophy. 

I. 

Gentlemen, — I  purpose  to  study  witli  you,  in  a  séries  of  four 
lectures,  some  imperfectly  known  parts  of  the  auatomical  and 
clinical  history  of  progressive  locomotor  ataxia.  The  clinical  point 
of  view,  especially,  is  that  from  which  I  intend  to  address  you  in 
thèse  conférences. 

The  task  of  the  clinical  observer  may  be  regarded  as  differing 
much  from  that  of  the  nosographer.  The  latter  concerns  himself 
chiefly  with  the  abstract  picture  of  diseases  ;  hence,  he  purposely 
neglects  anomalies — déviations  from  the  typical  standard — or  will- 
ingly  relegates  them  to  the  background. 

The  clinical  observer,  on  the  contrary,  lives  more  especially  on 


4        IMPOETANCE  OF  ANATOMICAL  EESEAECH. 

individual  cases  which  almost  always  présent  themselves  with 
peculiarities  that  separate  them  more  or  less  from  the  common  type. 
He  cannot  neglect  exceptional,  abnormal  cases,  for  tliese  are  the 
cases  which  chiefly  demand  the  exercise  of  his  sagacity. 

Now,  with  respect  to  the  former  class,  I  should  hâve  but  little 
to  add  to  the  classic  descriptions  which,  in  the  last  few  years,  hâve 
been  over  and  over  again  rehearsed,  and  which,  besides,  simply 
reproduce  with  some  variations  the  masterly  description  that  we 
owe  to  Dr.  Duchenne  (de  Boulogne) . 

On  the  other  hand,  the  anomalies  of  locomotor  ataxia,  the  dévia- 
tions from  the  normal  type,  offer  for  our  investigation  a  yet  vast 
field  which  is  far,  indeed,  from  having  been  explored  in  ail  its 
directions.  In  this  exposition  we  intend,  of  course,  to  make  use  of 
the  many  cases  which  are  subject  to  our  examination  in  this  hospital. 
They  will  enable  us  to  show  you,  along  with  the  ordinary  types, 
unexpected  combinations  which  hâve  been  little  or  not  at  ail 
studied,  and  which  yet  are  far  from  constituting  rare  cases. 

Without  neglecting  anatomical  investigation,  we  shall  hâve 
recourse  to  it  only  in  so  far  as  it  is  capable  of  throwing  light  on 
clinical  points  which  are  difficult  of  interprétation.  In  fact,  in  the 
présent  conférence  I  purpose  to  show  you,  with  respect  to  loco- 
motor ataxia,  what  advantage  a  clinical  observer  can  draw  from 
anatomical  researches  carried  out  in  a  certain  direction,  and  follow- 
ing  a  certain  method.  Let  us,  therefore,  make  ourselves  anatomists 
for  to-day  ;  examination  of  the  living  subject  will  then  résume  its 
rights. 

IL 

The  term  "  locomotor  ataxia  "  answers  to  a  dénomination  which 
is  altogether  symptomatic,  and,  for  some  time,  there  was  hésitation 
as  to  what  department  of  the  peripheral  or  central  nervous  System 
we  should  correlate  the  group  of  symptoms  which  this  term  désig- 
nâtes. Some  accused  the  brain  and  cerebellum,  others  the  spinal 
cord,  with  or  without  complicity  on  the  part  of  the  peripheral 
nerves.  Others,  in  short,  maintained  that  locomotor  ataxia  was  a 
neurosis,  and  held  that  the  lésion  of  the  nerve-centres,  discoverable 
on  autopsy,  was  only  produced  in  the  long  run  as  a  distant  but 
nowise  necessary  conséquence  of  prolonged  funclional  disturbance. 
Many  of  you  may  hâve  also  l;eard  this  thesis  developed  by  a  skil- 
fui  master.  Trousseau. 


SCLEROSIS    OF   POSTEEIOE    COLUMNS.  5 

They  based  their  opinion,  then,  upon  some  necroscopic  exami- 
nations,  reputedly  négative,  and  thèse  resultless  investigations 
made  a  more  striking  impression  on  the  mind,  because  they  had 
been  conducted  with  ail  the  appliances  of  the  most  délicate  research, 
and  by  compétent  masters  of  the  microscope.  But,  to-day,  those 
imaginary  facts  are  exploded.  The  method  was  insufïicient,  and  we 
believe  we  may  affirm  that,  in  the  very  first  period  of  locomotor 
ataxia,  even  when  the  disease,  still  at  its  outset,  is  only  marked  by 
lightning  pains,  easily  discernable  lésions  are  to  be  found  in  certain 
well-determined  parts  of  the  nervous  System — in  the  posterior 
columns  of  the  spinal  cord. 

I  hope  to  show  you,  gentlemen,  that  thèse  researches,  which  are 
so  minute  in  appearance,  only  require  care,  time,  and  the  applica- 
tion of  a  particular  method,  simple  enough  of  its  kind,  to  yield 
results  as  positive  as  those  furnished  by  the  most  elementary  process 
of  descriptive  anatomy — examination  with  the  naked  eye. 

III. 

But  this  point  you  should  remark  at  starting  :  if  it  be  true  that 
progressive  locomotor  ataxia  is  related,  as  has  been  said,  to  posterior 
sclerosis  as  "  the  shadow  to  the  body,"  you  must  not,  however, 
imagine  that,  in  calling  sclerosis  of  the  posterior  columns  by  the 
name  of  locomotor  ataxia,  you  possess  an  adéquate  définition. 

Assuredly  this  is  not  the  case,  and  I  hope  it  will  be  easy  for  me 
to  establish,  on  a  foundation  of  facts,  the  following  propositions, 
which  I  confine  myself,  for  the  moment,  to  stating  in  a  summary 
manner  : 

1°.  The  posterior  columns  are  sometimes  attacked  with  sclerosis, 
throughout  a  great  portion  of  their  extent,  without  symptoms  of 
ataxia  being  the  conséquence. 

2°.  Certain  lésions  of  the  spinal  cord,  originally  developed 
Gutside  the  posterior  columns,  may,  at  a  given  moment,  invade  them 
to  a  variable  height,  and  accidentally  produce  some  of  the  sym- 
ptoms of  ataxia,  which  I  would  willingly  term  tahetic  symptoms, 
but  we  hâve  not  really  hère  progressive  locomotor  ataxia. 

3°.  In  fact,  this  disease  is  evolved  with  an  array  of  symptoms 
following  each  upon  the  other  in  a  determinate  order,  which  is 
always,  or  nearly  always,  the  same.  It  is  a  disease  which  takes 
rank  apart,  self- dépendent.  The  lésion  with  which  the  symptoms 
aie   connected   occupies,   indeed,   the   posterior   columns,    but  it 


6  MAGEOSOOPIO    EXAMINATION. 

systematically  occupies,  in  those  columns,  a  fixed  and  circumscribed 
position,  which  is  always  the  same.  The  latter  point  is  that  which 
we  shall  first  seek  to  demonstrate. 

IV. 

Let  us,  then,  enter  at  once  upon  the  question  of  pathological 
anatomy.  The  anatomical  study  of  spinal  scléroses  includes,  gene- 
rally  speaking,  as  you  are  aware — \°,  examination  with  the  naked 
eye  ;  a°,  microscopical  examination  of  fresh  sections  and  of  sections 
hardened  by  différent  methods. 

We  shall  be  brief  with  respect  to  the  results  of  macroscopic 
investigation  in  ataxia,  for  it  is  manifestly  insufficient,  stricken  with 
sterility  from  the  outset.  In  récent  cases,  in  fact,  it  does  not  note 
any  altération,  and  in  cases  of  old  standing  it  gives  us  the  means 
neither  of  determining  exactly  the  seat  of  the  lésion,  nor  its  nature. 
It  simply  informs  us  that  there  is  a  grey  induration.  This  is  ail, 
and  it  is  not  enough. 

Nevertheless  we  should  not  forget  that,  in  spite  of  its  imperfec- 
tions, we  owe  to  this  mode  of  examination  the  discovery  of  sclerosis 
of  the  posterior  columns.  And,  if  I  notice  this  fact  in  passing,  it 
is  because  this  was  an  entirely  Prench  conquest,  which  dates  from 
the  great  anatomo-pathologic  epoch  inaugurated  by  Bayle  and 
Laennec,  and  continued  by  Cruveilhier.  In  1827,  Hutin  showed 
to  the  Société  Anatomique  a  spécimen  of  gelatiniform  degeneratiou 
of  the  posterior  columns.  Then  foUowed  Monod  and  Ollivier 
(d'Angers).  But,  in  thèse  communications  the  symptoms  could 
not  be  set  face  to  face  with  the  lésions  ;  hence,  thèse  cases  excited 
little  attention  except  as  anatomical  curiosities.  Not  until  a  later 
day  was  the  connection  successfully  made  between  grey  induration 
of  the  posterior  columns  and  the  symptoms  of  ataxia, — definitely 
coôrdinated  by  Dr.  Duchenne  (de  Boulogne), — and  then  it  was 
shown  that  the  disease,  at  first  considered  as  exceptional,  is  in 
reality  very  common. 

Besides  the  altérations  of  the  posterior  columns,  macroscopic 
anatomy  has  revealed — 1°,  altération  of  the  posterior  roots  (atropliy); 
2°,  that  of  the  méninges  (spinal  posterior  meningitis)  ;  3°,  that  of 
difiierent  cérébral  nerves,  and,  among  others,  of  the  optic,  the  oculo- 
motor,  and  the  hypoglossal  nerves  (atrophy  and  grey  degeneratiou). 
Let  us  also  say  that,  speaking  generally,  it  has  allowed  us  to  note 
that  the  lésions  are  more  marked  in  the  cervical  région,  when  the 


DESIDEEATA.       HISTOLOGIO    RESEARCH.  7 

symptoms  predominate  in  the  upper  extremities,  and  in  the  lumbar 
région  in  the  common  form,  that  is  to  say,  when  the  morbid  phe- 
nomena  are  especially  manifest  in  the  lower  extremities.  Let  us 
add,  in  conclusion,  that  the  invasion  of  the  latéral  columns  by  the 
grey  induration  did  not  escape  notice  by  this  method  of  exami- 
nation. 

Still,  I  must  repeat  that  macroscopic  anatomy  furnished  no 
information  with  respect  to  the  state  of  the  grey  substance,  to  the 
exact  localisation  of  the  lésion,  to  the  genesis  and  nature  of  the 
morbid  process,  nor  to  many  another  point  besides. 

V. 

The  study  of  the  spinal  cord,  by  means  of  magnifying  lenses,  is 
alone  able  to  supply  the  desiderata  which  we  hâve  just  noticed. 
Its  methods  are,  besides,  applicable  from  différent  points  of  view, 
It  may,  in  the  first  place,  propose  to  itself  to  go  back  even  to  the 
anatomical  éléments  themselves,  and  to  seek  out  the  changes  which 
they  undergo  in  the  différent  phases  of  the  morbid  process.  It 
was  thus  it  became  recognised  that  the  lésion  of  the  posterior 
columns,  in  progressive  locomotor  ataxia,  is  one  of  the  forms  of 
sclerosis  of  the  nerve-centres. 

Sclerosis  or  grei/  induration  of  the  nerve-centres  corresponds, 
you  hâve  not  forgotten,  to  one  of  the  modes  of  primary  chronic 
inflammation.  One  of  its  most  salient  features  is  the  hyperplasia, 
with  fibrillary  metamorphosis  of  the  neuroglia,  taking  place  at  the 
expense  of  the  nerve-elements,  or  proceeding,  at  least,  on  parallel 
lines  with  the  destruction  of  those  éléments. 

I  will  not  insist  hère  on  showing  that,  as  regards  its  histological 
characters,  grey  induration  in  locomotor  ataxia  differs  in  nothing 
essential  from  what  it  is  in  disseminated  sclerosis  or  in  symme- 
trical  sclerosis  of  the  latéral  columns,  for  instance.  That  question 
appears  to-day  definitely  set  at  rest,  and  nobody  now  admits,  I 
think,  that  only  simple  atrophy  is  présent,  as  some  authors, 
amongst  them  Herr  Leyden,  maintained.  Spinal  meningitis,  which 
so  frequently  coexists  with  sclerosis  of  the  posterior  fasciculi,  in 
ataxia,  and  which,  in  such  cases,  always  shows  itself  exactly  limited 
to  the  surface  of  thèse  fasciculi,  would  supply,  if  necessary,  a  new 
argument  in  favour  of  the  irritative  nature  of  the  altération. 

But  there  is  a  point  on  which  it  seems  impossible  to  pronounce 
judgment  at  présent  in  a  definite  manner  : — What  is  the  original 


8  MIOROSCOPICAL    TOPOGRAniIC    ANATOMY. 

focus  of  tliis  irritative  lésion  ?  Is  it  tlie  neuroglia  ?  Is  it,  on  tlie 
contrar}',  the  nerve-element  ?  I  confess  that,  considering  liow  tlie 
altération  remains,  in  some  sort,  systematically  confnied  within  the 
area  of  tlie  posterior  fasciculi,  wliose  limits  it  does  not  cxceed,  ex- 
cept  in  exceptional  circumstances,  I  cannot  help  being  strongly 
inclined  to  the  latter  hypothesis.  Parenchymatous  irritation  would, 
therefore,  be  the  initial  fact  ;  interstitial  irritation  would  be  secon- 
dary.  I  shall  perhaps  find,  as  we  proceed,  an  occasion  for  pointing 
ont  to  jour  attention  some  otlier  data  whicli  yield  support  to  this 
view  of  tbe  matter. 

YI. 

The  study  of  the  spinal  cord,  by  means  of  magnifying 
glasses,  may  also,  as  we  mentioned,  be  conducted  from  another 
point  of  view.  There,  in  fact,  exists  a  kind  of  microscopical  topo- 
graphie anatomy,  the  object  of  which  is  to  examine  the  parts  in  their 
natural  relations,  without  either  destruction  or  dilaceration.  The 
point  is  then,  principally,  to  détermine,  in  an  exact  way — within  the 
area  of  the  white  columns  or  in  the  différent  departments  of  the 
grey  substance — what  is  the  extent,  the  configuration,  the  précise 
allocation  of  the  changes,  and  to  trace  their  mode  of  extension 
when,  flowing  ont  from  their  primai  source,  they  spread  to  adjacent 
régions  or  even  to  distant  parts. 

To  this  mode  of  investigation  we  owe,  if  I  mistake  not,  a  con- 
sidérable portion  of  the  progress  recently  accomplished  in  the 
history  of  chronic  spinal  diseases,  which  for  such  a  length  of  time 
lias  been  almost  undecipherable.  This  resuit,  indeed,  it  was  easy 
to  foresee.  Experiments  on  animais,  notwithstanding  the  com- 
paratively  coarse  method  employed,  had  yet  suflîced  to  place  beyond 
doubt  the  fact  that,  in  this  slender  cord,  which  is  called  the  spinal 
marrow,  there  exists,  even  in  the  white  columns  alone,  several 
régions  corresponding  to  as  many  organs,  the  functions  of  which 
are  wholly  distinct,  notwithstanding  the  analogy  of  their  composi- 
tion. Thus,  the  latéral  fasciculi  are,  physiologically,  quite  distinct 
from  the  posterior  fasciculi.  In  the  grey  substance  itself  we  hâve 
grounds,  as  you  are  aware,  for  laying  down,  from  this  point  of 
view,  a  certain  number  of  more  or  less  distinctly-divided  districts. 

Pathology,  in  its  turn,  came  to  confirm,  in  a  gênerai  manner, 
thèse  data  in  showing  that  a  lésion,  limited  to  such  or  such  a  de- 
partment,  is  manifested  each  time  by  a  particular  group  of  sym- 


MICROSCOPICAL  TOPOGRAPHIC  ANATOMY.         9 

ptoras  ;  but  it  was  destined,  in  our  own  dajs,  with  tlie  help  of  new 
means  of  anatomical  study,  to  go  further  and,  on  many  points^,  to 
advance  beyond  expérimental  research.  Even  in  tlie  hands  of  the 
raost  skilful  expérimenter  could  the  latter  method  ever,  in  an 
organ  so  délicate  and  so  difficult  of  access  as  the  spinal  cord,  déter- 
mine lésions  limited,  for  instance,  to  certain  groups  of  nerve-cells, 
or  to  certain  fasciculi  of  fibres  ?  It  is  lawful  to  doubt  it.  Disease, 
on  the  other  hand,  daily  produces  such  lésions,  and  the  topographie 
anatomy  of  the  cord  enables  us  to  recognise  them  with  the  greatest 
précision.  It  permits  us  to  observe  what  group  of  nerve-cells, 
what  fasciculi  of  nerve-fibres  hâve  been  irritated,  atrophied,  or 
destroyed.  Face  to  face  with  this  délicate  anatomy,  place  observa- 
tions carefully  coUected  (in  which  the  analysis  of  the  symptoms 
will  be  ail  the  more  complète  and  profitable  when  made  by  the 
light  of  preliminary  physiological  and  anatomical  knowledge),  and 
you  shall  hâve  in  your  hands  the  conditions  of  an  experiment 
taking  place  spontaneously,  as  it  were,  and  taking  place  in  the 
human  subject,  which,  as  regards  this  matter,  is  an  inestimable 
advantao'e. 

We  may  say  that,  to-day,  thanks  to  researches  directed  m  this 
spirit,  the  history  of  a  good  number  of  spinal  affections,  whose 
pathogeny  had  previously  remained  in  deep  obscurity,  has  been 
cleared  up  by  an  unexpected  light.  We  hâve  learned,  for  instance, 
that  the  paralysis  of  children,  termed  essential,  is  due  to  a  myelitis 
pystematically  confined  to  quite  a  small  department  of  the  grey 
substance  of  the  cord — the  région  of  the  anterior  cornua.  We 
know  also  that  a  majority  of  the  cases,  designated  clinically  by  the 
name  of  progressive  muscular  atrophy,  are  to  be  referred  to  an 
altération  occupying  the  same  situation,  but  in  which  lésion  of  the 
nerve-cells  takes  place,  not  in  an  acute  manner,  but  after  a  chronic 
progressive  fashion.  Thèse  instances  might  readily  be  augmented, 
but  we  must  limit  ourselves,  and  now  return  to  the  principal  object 
of  our  study. 

VII. 

We  hâve  endeavoured,  gentlemen,  during  the  course  of  the  last 
two  years,  to  apply  the  method  we  are  lauding  to  the  revision  of  the 
anatomical  data  which  concern  progressive  locomotor  ataxia.  The 
results  acquired  to  science,  although  still  imperfect  in  some  respects, 
appear  nevertheless  worthy  of    being  placed  before  you.      They 


10 


SCLEROSIS  OF   GOLL  S    COLTJMNS. 


are,  for  the  most  part,  due  to  the  researches  conducted  iii  accord- 
ance  with  my  advice  by  M.  Pierret,  assistant  in  my  wards.  Thèse 
labours  hâve  been  made  the  subject  of  two  memoirs  published  in 
the  '  Archives  de  Physiologie.'  '  1  shall  apply  myself  only  to  point- 
ing  out  to  you  the  most  important  points  which  hâve  there  been 
elucidated. 

I  hâve  given  you  to  understand  that  the  lésion  of  the  posterior 
columns  of  the  cord,  to  which  belong  the  symptoms  of  locomotor 
ataxia,  do  not  indiscriminately  affect  ail  the  parts  of  thèse  fasciculi, 
but  strictly  occupy  certain  régions  which  it  now  behoves  us  ta 
détermine. 


YlG.    I. 


TlG.    2. 


Sclerosis  limited  to  the  médian  or  Goll's  columns. 
TiG.  I. — Cervical  région.  Tig.  2. — Dorsal  région. 

It  liad  long  been  remarked  that  fasciculated  and  ascending 
sclerosis  of  the  posterior  fasciculi,  such  as  is  seen,  for  instance,  in 
the  cervical  enlargement,  in  cases  where  the  cord  is  compressed  in 
a  point  of  the  dorsal  région,  owing  to  Pott's  disease,  does  not 
resuit,  except  under  spécial  circumstances,  in  the  production  of 
ataxic  symptoms  (motor  incoordination  and  lightning  pains)  in  the 
upper  extremities.  Now,  this  consécutive  sclerosis  aflFects  exclusively, 
in  such  cases,  the  médian  or  GolPs  columns.  Hence,  that  fact 
had  already  made  it  seem  likely  that,  in  ataxia,  GolFs  columns 
should  be  excluded  from  ail  participation  in  the  production  of  the 
essential  symptoms  (figs.  i,  2,  3,  4,  also  Plate  I,  fig.  i). 

Careful  scrutiny  of  the  lésions  présent  in  the  cervico-dorsal 
régions,  in  cases  of  generalised  locomotor  ataxia  (that  is,  ataxia 

'  Pierret,  "Notes  sur  la  sclérose  des  cordons  postérieurs  dans  l'Ataxie 
locomotrice  progressive,"  '  Arcliives  de  Physiologie,'  1872,  p.  364.  "  Notes  sur 
un  cas  de  sclérose  primitive  du  faisceau  médian  des  cordons  postérieurs,'  ibid.^ 
1873,  P-  74. 


SOLEROSIS    OF   EXTBENAL   BANDS.  11 

affecting  both  the  upper  and  lower  extremities),  confirmed  tliis 
opinion.  In  cases  of  this  kind,  besides  the  sclerosis  of  Goll's 
column — which  is  nearly  always  présent — you  remark  two  slender 
grey  bands  which  were  detected  with  the  naked  eye  and  pointed  out^ 
at  the  time,  by  M.  Vulpian  and  myself. 

FiCt.   3.  PiG.    4. 


Sclerosis  limited  to  GoU's  columns. 
FiG.  3. — Section  made  at  the  i2th  dorsal  vertebra. 

FiG.  4. — Superior  portion  of  the  lumbar  enlargement.      (Thèse  6gures  are 
borrowed from the  'Archives  de  Physiologie.') 

Thèse  bands,  seen  on  the  surface  of  the  cord,  appear  to  occupy 
the  posterior  collatéral  furrows,  and  the  most  internai  of  the  sensi- 
tive  roots  seem  to  émerge  from  them  (PI.  I,  fig.  2).  On  transverse 
sections  they  show  themselves  under  the  form  of  two  grey  tracts, 
running  from  before  backwards  and  slightly  from  without  inwards  ; 
thèse  tracts  are  separated  from  GolPs  columns  on  the  inner  side, 
and  from  the  posterior  grey  cornua  on  the  outer  side,  by  thin  bands 
of  white  substance  presenting  the  normal  character.  Now, 
gentlemen,  it  follows,  from  numerous  observations  which  we  hâve 
collected,  along  with  M.  Pierret,  that  the  sclerosed  tracts  in  ques- 
tion are  never  met  with  save  in  cases  in  which^  during  life,  the  upper 
extremities  exhibited  tabetic  symptoms.  Such  tracts  exist  only  on 
the  right  side  of  the  cord,  or  they  predominate  there,  when  the 
right  upper  extremity  was  alone  or  chiefly  afFected.  The  contrary 
happens  when  the  symptoms  predominated  in  the  left  upper  extre- 
mity. Moreover,  in  ail  the  cases  in  which  the  superior  extremities 
had  remained  perfectly  free  the  tracts  were  completely  absent.  The 
présence  of  tabetic  symptoms  therefore  appears,  as  you  see,  to  be 
intimately  connected  witL  the  existence  of  thèse  latéral  sclerous 
bands. 


13  SCLEEOSIS    OP    EXTERNAL    BANDS. 

But  there  was  still  wanting,  to  complète  tlie  démonstration,  a 
case  in  which  sclerosis  of  GoU's  columns  should  be  completely 
absent  from  tlie  brachial  enlargemeut,  althougli  ataxic  symptoms 
had  been  exhibited  in  the  upper  extremities.  This  case,  at  last, 
was  found  ;  it  was  tliat  of  a  patient,  named  Moli,  whose  history  lias 
been  detailed  in  full  in  one  of  the  memoirs  of  M.  Pierret.  Motor 
incoordination  and  shooting  pains  had  existed,  to  a  high  degree,  in 
both  the  upper  extremities  of  this  woman,  nevertheless  the  scierons 
lésion  was  only  represented,  at  the  autopsy,  in  the  dorso-cervical 
région  of  the  cord  by  two  thin  greij  bands.  The  médian  fasciculus 
was  exempt  from  ail  altération  (PI.  I,  iig.  3). 

It  manifestly  follows  from  the  foregoing  that,  so  far  as  the  supe- 
rior  extremities  are  concerned,  the  lésion  of  GolFs  columns  cannot 
claim  any  part  in  the  production  of  tabetic  symptoms.  What,  then, 
can  be  the  reason  of  the  habituai  existence  of  this  lésion  in  ataxia  ? 
M.  Pierret  lias  expressed  the  opinion  that  we  hâve  hère  a  pheno- 
menon  analogous  to  that  which  détermines  ascending  médian  fascicu- 
lated  sclerosis,  consécutive  on  partial  myelitis;  according  to  this  view, 
the  lésion  of  Goll's  columns  would  not  be  produced  in  the  cervical 
région,  in  ataxic  patients,  except  in  cases  where  the  sclerosis  is  very 
markedly  présent  in  the  dorso-lumbar  région.  I  fuUy  endorse  this 
opinion,  and  I  hâve  not  hitherto  met  with  any  fact  to  contradict  it. 

What  we  hâve  said  with  respect  to  the  upper  extremities  applies 
equally  to  the  lower  extremities,  The  case  of  Moli  is  a  proof  of 
this.  In  her  case  locomotor  ataxia  showed  itself  in  its  generalised 
form.  Lightning  pains  and  motor  incoordination  affected  the 
lower  as  well  as  the  upper  extremities,  and  yet  the  lésion  of  the 
médian  columns  was  absent  from  the  lumbar  région  just  as  inuch  as 
irom  the  cervical  enlargement.  The  two  self-same  latéral  bands,  to 
which  I  called  your  attention,  were  alone  engaged  throughout  the 
whole  lengtli  of  the  cord,  and  to  thèse,  in  her  case,  sclerosis  of  the 
posterior  fasciculi  was  restricted. 

You  see,  gentlemen,  that  the  sclerous  lésion  of  the  latéral  bands 
is,  in  short,  the  only  essential  auatomical  fact  in  progressive  loco- 
motor ataxia.  Sclerosis  of  the  médian  columns,  on  the  contrary, 
is  only  a  chance,  an  incidental,  and  probably  a  consécutive  fact. 

The  foregoing  facts  eut  short,  as  you  must  hâve  remarked, 
certain  récriminations  which  sceptics  hâve  taken  pleasure  in  raising 
against  the  results  furnished  by  pathological  anatomy.  They 
accuse  it  of  being  delusive  and  faithless,   because  it  sometimes 


RELATION    BETWEEN    LESIONS    AND    SYMPTOMS.  13- 

exhibits  ataxia  without  posterior  sclerosis,  and  sometimes  posterior 
sclerosis  without  ataxia. 

The  truth  is  that  sclerosis  of  the  latéral  bands  of  the  posterior 
fasciculi  is  the  only  constant  lésion  in  locomotor  ataxia  :  that  lésion 
exists^  gentlemen,  in  every  epoch  of  the  disease,  but  one  must 
know  how  to  seek  for  it  where  it  is  to  be  found. 

YIII. 

Erom  what  précèdes,  you  understand,  we  hâve  grounds  for 
establishing  two  very  distinct  forms,  in  posterior  sclerosis,  which 
may  be  manifested  separately,  independent  of  each  other,  or  which, 
on  the  contrary,  may  be  combined.  One  of  thèse  forms  may  be 
designated  by  the  name  of  médian  sf/stematic  fasciculated  sclerosis, 
or  sclerosis  of  GolVs  columns  ;  it  exists  sometimes  as  a  consécutive 
ajffection  (consécutive  ascending  sclerosis),  sometimes  as  a  primary 
affection.  We  know  not,  up  to  the  présent  time,  what  peculiar 
symptoms  are  counected  with  this  form  of  posterior  sclerosis.  The 
other  form,  latéral  fasciculated  sclerosis  of  the  posterior  column,  or 
sclerosis  of  the  external  hands,  holds  under  its  dominion  the  tahetic 
symptoms  ;  like  the  first  named,  it  may  be  deuteropathic,  or,  on  the 
contrary,  protopathic.  This  form  is  nothing  other  than  the 
anatomical  substratum  of  progressive  locomotor  ataxia. 

I  think  it  useful  to  enter  upon  some  new  détails  in  order  to 
make  it  clearly  manifest  that  this  latéral  fasciculated  sclerosis  is,  in 
reality,  the  fondamental  anatomical  fact  in  ataxia.  It  seems  to  us 
possible  to  establish,  in  the  first  place,  that  it  is  found  from  the 
outset  of  the  disease,  even  at  the  period  when  lightning  pains 
form,  by  themselves  alone,  the  entire  clinical  picture,  without  being 
accompanied  by  motor  incoordination  :  then,  it  may  exist  alone, 
no  altération  being  yet  discoverable  in  Goll's  columns.  This  fact, 
we  believe,  bas  been  placed  beyond  a  doubt,  in  several  cases  where 
death,  supervening  from  a  complication,  came  to  prematurely 
arrest  the  development  of  the  disease.  As  an  illustration,  I  may 
quote  the  case  of  the  patient  Âllard,  noted  by  M.  Pierret.  Under 
such  circumstances  it  must  not  be  forgotten  that  the  naked-eye 
and  even  an  ill-conducted  microscopic  examination  would  be  often 
powerless  to  discover  the  spinal  altération  which  a  methodical 
study  of  properly  prepared  and  hardened  sections  alone  is  sufificient 
to  reveal, 

It  would  resuit  from  this  datum  that,  contrary  to  the  assertions  of 


14  EELATION    BETWEEN    LESIONS  AND   SYMPTOMS. 

standard  authorS;,  who  make  the  sclerosis  of  ataxia  begin  bj  the 
médian  parts,  in  the  vicinity  of  the  méninges,  it  really  commences 
by  the  région  of  the  latéral  bands.  We  should  add  that,  according 
to^  our  observations,  at  this  period  of  the  disease  the  posterior  spinal 
roots  do  not  yet  generally  présent  any  appréciable  altération  ;  and, 
finally,  that  the  latéral  scierons  bands  are  then  very  narrow,  reduced, 
so  to  say,  to  slender  linear  strips. 

There  is  reason  to  hope  that,  with  the  help  of  a  great  number  of 
■observations,  very  varied  as  regards  the  symptoms,  and  noted  at 
différent  periods  of  the  disease,  it  will,  sooner  or  later,  be  possible — 
by  the  careful  comparison  of  clinical  facts  with  microscopic  results 
— to  identify  the  mode  of  progressive  invasion  of  the  lésion, 
whether  it  proceeds  from  within  outwards,  or  from  without  inwards, 
and,  at  the  same  time,  to  détermine  the  différent  régions  which 
give  rise  to  the  symptoms  that  successively  appear. 

The  foUowing  are  some  results  towards  which  we  hâve  been  led, 
so  far,  by  the  researches  instituted  with  that  aim.  The  scierons 
bands,  which  are  very  slender,  very  narrow,  so  long  as  the  symp- 
tomatology  embraces  only  the  lightning  pains,  enlarge  both  out- 
wardly  and  inwardly  when  motor  incoordination  is  added  to  thèse. 
If  a  very  marked  ansesthesia  hâve  existed,  the  posterior  horns  of 
the  grey  substance  are  in  their  turn  invaded  by  altération,  and 
at  the  same  time  we  remark  a  great  number  of  nerve-tubes  affected 
by  atrophy  in  the  posterior  roots.  Finally,  the  paretic  or  paralytic 
symptoms,  with  or  without  contracture,  which  sometimes  come  to 
be  added  to  the  incoordination,  generally  at  an  advanced  period  of 
the  évolution,  answer  to  an  invasion  of  the  posterior  part  of  the 
latéral  columns.  With  respect  to  the  habituai  extension  of  the 
latéral  sclerosis  to  the  médian  columns,  we  hâve  not  hitherto 
found  that  it  added  anything  whatever  to  the  ordinary  symptoms  of 
the  disease. 

The  progressive  extension  of  the  sclerous  lésion  beyond  the  foci 
which  it  occupied  at  the  outset,  may,  besides,  proceed  in  two  prin- 
cipal directions.  We  hâve  just  seen  how,  in  the  transverse  plane, 
it  takes  place,  either  outwards,  towards  the  posterior  cornua  of  the 
grey  substance,  or  inwards,  towards  the  médian  columns.  Verti- 
cally  considered,  it  extends  progressively,  at  least  under  ordinary 
circumstauces,  from  the  dorso-lumbar  région  towards  the  cervical 
région,  still  preserving  the  arrangement  of  latéral  bands  ;  whilst,  at 
the  same  time,  the  médian  columns  usually  become  the  seat  of  an 


LOCOMOTOR   ATAXIA  AND    MUSCULAE   ATROPHT.  15 

ascending  consécutive  sclerosis.  The  prolongations  of  the  latéral 
bands  may,  besides^  be  followed  into  the  bulbus  rachidicus,  where 
they  occupj  the  région  of  the  restiform  bodies. 

IX. 

It  is  not.  devoid  of  interest  to  investigate  whether,  as  seems  a 
priori  very  probable,  this  localisation  of  the  scierons  lésions  in  a 
determiuate  région  of  the  posterior  columns,  which  may  be  termed 
the  région  of  external  lands,  may  not  be  correlated  with  a  particular 
anatomical  arrangement.  Most  assuredly  sach  an  arrangement 
exists.  In  fact,  the  bands  in  question  correspond  exactly  to  the 
intramedullary  distribution,  described  by  Stilling,  Clarke,  and 
KoUiker,  of  those  of  the  nerve-filaments,  emanating  from  the  pos- 
terior spinal  roots,  which  are  commonly  designated  by  the  name  of 
internai  radicular  fascicles  (KoUiker).  But  thèse  nerve-filaments 
do  not  alone  constitute  the  bands,  for  the  sclerous  lésion  is  found 
quite  as  well  marked  in  the  space  which  séparâtes  the  points  of 
insertion  of  the  posterior  roots  as  at  thèse  points  themselves.  This 
renders  it  very  probable  that,  besides  the  internai  root  bundles, 
there  exist,  in  this  région  of  the  posterior  columns,  other  bundles 
of  fibres  which,  doubt^less,  constitute  vertical  connections  between 
différent  parts  of  the  spinal  cord.  Thèse  fibres  would  serve  for  the 
coordination  of  the  movements  of  the  extremities  ;  at  ail  events 
we  know  positively,  from  what  précèdes,  that  lésion  of  them  pro- 
duces incoordination,  whilst  lésion  of  the  bundles  of  fibres  which 
constitute  the  médian  columns  does  not  appear  to  bave  this  effect. 

The  relation  which  exists  between  the  course  of  the  internai  root 
bundles  and  the  seat  of  the  lésions  of  the  ataxia  will  doubtless  help 
us  to  understand  the  appearance  of  certain  complications  which  are 
observed  in  this  disease.  I  shall  confine  myself  to  one  example. 
You  know  that  it  is  not  rare,  in  the  course  of  locomotor  ataxia,  to 
notice  the  supervention  of  muscular  atrophy,  sometimes  partial, 
sometimes,  on  the  contrary,  more  or  less  generalised. 

The  anatomical  reason  of  this  complication  seems  to  us  to  be 
demonstrated  in  the  following  observation,  The  case  is  that  of  the 
patient  Moli,  to  which  allusion  bas  already  been  made.' 

The  ataxia,  characterised  by  acute   lightning  pains,  and  very 

^  Pierret,  "  Sur  les  altérations  de  la  substance  grise  de  la  moelle  épinière 
dans  l'ataxie  locomotrice  considérées  dans  leur  rapports  avec  l'atrophie  mus- 
culaire."   'Archives  de  Physiologie,' 1870,  p.  590. 


16 


LESIONS    OF    CELLS  OF    GRE  Y    SUBSTANCE. 


marked   motor-incoordination,    had   been   long   manifesta   in   this 
woman's   case^  when  muscular  atrophy  supervened,  wliich  made 


TiG.  5. — A.  Posterior  roots.  B.  Internai  radical  filaments,  and  sclerosis 
limited  to  tlieir  course.  C.  lliglit  anterior  cornu  atrophied.  (Thi& 
figure  is  taken  from  tlie  'Archives  de  Physiologie.') 

rather  rapid  progress  under  our  eyes,  but  confined  itself,  in  a  very 
definite  manner,  to  the  upper  and  lower  extremities  of  tlie  right 
side.  I  could  not  bring  myself  to  look  upon  tliis  complication  as 
a  fortuitous  coincidence,  and  I  stated^  as  my  opinion,  that  the  amyo- 
tropby  arose,  in  this  case,  from  the  extension  of  the  scierons  lésion 
of  the  posterior  columns  to  the  anterior  grey  cornu  of  the  right  side 
(fig.  5).  The  patient  having  succumbed  to  an  intercurrent  affection, 
the  autopsy  fully  justified  my  forecast.  In  the  dorsal,  as  well  as  in 
the  cervical  région  of  the  spinal  cord,  the  grey  cornu  of  the  right 
side  was  evidently  atrophied.  The  large  motor  cells  showed  great 
altération  ;  those,  in  particular,  which  formed  the  external  group, 
had  for  the  most  part  disappeared  to  give  place  to  an  islet  of 
sclerosis.  Now,  we  know  that,  according  to  Kolliker's  description, 
a  certain  number  of  the  nerve-filaments  which  compose  the  internai 
root  bundles  proceed  towards  the  anterior  grey  cornua,  and  may  be 
followed  to  this  external  group  of  motor  nerve-cells.  It  is,  pro- 
bably,  by  means  of  thèse  nerve  tubes  that  the  irritative  process, 
primarily  developed  in  the  posterior  columns,  was  propagated  to 
the  extremities  of  the  anterior  grey  substance,  and  there  determined 
the  lésions  which  préside  over  the  development  of  spinal  amyo- 
trophy. 


LESIONS    OF   CELLS    OF    GRET    SUBSTANCE.  17 

Time  presses,  and  I  cannot  further  prolong  my  remarks.  I 
venture  to  hope,  however,  that  the  détails  which  I  hâve  just  dis- 
cussedj  will  suffice  to  enable  you  to  appreciate  tlie  advantage  whieh 
•clinical  observation  may  dérive  from  délicate  anatomical  studies, 
conducted  in  accordance  with  the  method  I  désire  to  recommend 
io  your  attention. 


TOL.   II. 


LECTURE  II. 

RETROGRADE  ACTION  IN  SPINAL  DISEASES  :  ITS  RELATION 
WITH  SCLEROSIS  OF  THE  POSTERIOR  COLUMNS;  LIGHT- 
NING  PAINS  AND  GASTRIC  CRISES. 

SuMMARY. — Relations  between  tlie  internai  radicular  nerve-fila- 
ments  and  the  groups  of  multïpolar  nerve-cells  qf  the  anterior 
cornua.  Récurrent  or  rétrograde  symptoms.  Scier osîs  of  the 
posterior  médian  bitndles  ;  propagation  of  tlie  sclerosis  ta  the 
latéral  hands  of  the  posterior  columns. 

Classical  type  of  progressive  locomotor  ataxia.  Prodromic 
period  of  shooting  pains.  Period  of  estaèlished  disease,  or 
of  tabetic  incoordination.     Paralytic  period. 

Lightning  pains.  Their  varieties  ;  boring  pains  ;  lancinating 
pains;  constrictive  pains.  Symptomatic  shooting  pains  in 
disseminated  sclerosis,  progressive  gênerai  paralysis  and 
chronic  alcoholism,  fisceral  symptoms:  vesical,  urethral 
pains  ;  rectal  tenesmus. 

Gastric  crises;  spécifie  characters.  Dnration.  Abortive- 
cases  of  locomotor  ataxia. 

1 

Gentlemen. — Some  of  my  auditors  did  me  the  honour,  at  the 
conclusion  of  my  last  lecture,  to  ask  me  for  some  explanations 
as  to  the  connection  wliich  appears  to  exist  between  the  internai 
radicular  nerve-filaments,  issuing  from  the  posterior  roots,  and  the 
external  group  of  multipolar  nerve-cells  of  the  anterior  cornua.  I 
am  led  to  reply  that,  generally  speaking,  we  cannot,  in  the  actual 
state  of  science,  as  yet  lay  down  anything  absolutely  as  regards  the 
anatomical  relations  which  may  be  formed,  by  means  of  the  cellular 
prolongations,  either  from  cell  to  cell,  or  between  the  cells  and  the 
nerve-tubes,  whether  of  the  anterior  roots,  or  of  the  posterior  roots. 
Hère,  in  a  few  words,  is  what  the  most  récent  researches  teach 
upon  the  subject. 

Among  tlie  prolongations  which,  varying  in  number,  proceed 
from  the  great  nerve-cells  of  the  anterior  cornua,  there  is  one. 


NERVE-FIBRE    NET.  19 

as  you  are  aware,  vvhicli  in  every  cell  is  distinguislied 
froin  the  others  by  certain  well-marked  anatomical  cliaraeters. 
Very  fine,  very  slender  at  its  origin  from  tlie  cell,  it  gradually 
thickens,  without  ramifying,  and  soon  acquires  the  histological 
cliaraeters  of  a  nerve-tube.  It  is  tliis  prolongation  which  Deiters 
made  known,  under  the  name  of  JSIervenforsatz.  AU,  or  nearly  ail 
of  thèse  nerve-jjrolong citions,  according  to  Gerlach,  proceed  evidently 
towards  the  anterior  extremity  of  the  anterior  cornu,  where  they 
seera  to  enter  into  connection  with  the  radicular  filaments,  from 
which  arise  the  motor  spinal  roots.  As  to  the  other  cell-prolonga- 
tions  which,  to  distinguish  them  from  the  foregoing,  are  called 
Protoplasmaforsatze  (protoplasm  prolongations),  they  branch  out 
soon  after  leaving  the  cell,  and  the  iamifications  thus  produced 
branch,  again  and  subdivide,  almost  infinitely,  as  it  were,  so  as  to 
constitute  what  Herr  Gerlach  calls  the  nerve-fibre  net  {Nervenfaser- 
netz).  It  is  through  the  médium  of  this  net,  and  not  in  a  direct 
manner,  that  the  posterior  radicular  bundles  are  supposed  to  enter 
into  connexion  with  the  nerve-cells  of  the  anterior  cornua.i  There 
is  a  wide  différence  between  thèse  somewhat  vague  data  and  the 
almost  mathematical  précision  with  which  certain  authors  make  the 
nerve-cells  communicate  eitlier  between  themselves,  or  with  the 
nerve-filaments  of  the  anterior  and  posterior  roots.  But,  it  behoves 
us  never  to  confound  problematic  with  real  anatomy  ;  they  are 
whoUy  différent  things. 

II. 

There  is  another  point  relating,  this  time,  to  the  pathological 
anatomy  and  physiology  of  the  posterior  columns,  which  want  of 
time  caused  me  to  omit  mention  of,  and  yet  which,  I  believe, 
deserves  to  be  discussed. 

I  bave  to  remind  you,  gentlemen,  that  in  cases  of  ascending 
degeneration,  consécutive  on  a  partial  lésion  of  the  dorsal  cord, 
(partial  myelitis,  either  primary  or  connected  with  Pott's  disease,  or 
tumours  compressing  the  dorsal  cord),  the  posterior  columns  are 
affected  with  sclerosis,  throughout  their  whole  height  up  to  the 
vicinity  of  the  bulbus  ;  and  yet,  in  cases  of  this  kind  the  ataxic 
symptoms  are  absolutely  absent  from  the  superior  extremities,  at 
least  in  the  immense  majority  of  cases. 

There  are^  nevertheless,  exceptions  to  the  rule,  and  this  leads  me 
'  Gerlacb,  in  '  Stricker's  Handbuch,'  t.  ii,  p.  683. 


20  EECURRENT    SYMPTOMS.       DISTANT   ACTION. 

to  speak  a  few  words  aboat  wliat  we  may  call,  in  spinal  pathology, 
the  récurrent  or  rétrograde  st/mptoms — symptoms  whicli  were  well 
known  to  Marshall  Hall,  who  referred  them,  without  further  expia- 
nation,  to  a  rétrograde  action} 

If  I  mistake  not,  M.  Louis  2  was  the  first  author  who  seems  to 
hâve  been  struck  by  the  existence  of  facts  of  this  nature.  A 
patient,  suCFering  from  caries  of  the  dorsal  vertébrée,  had  exhibited, 
besides  paraplégie,  complète  paralysis  with  contracture  of  the 
upper  extremities.  Still,  the  autopsy  showed  that  the  dorsal  cord 
alone  had  been  softened  in  a  portion  of  its  extent.  Cases  of  this 
Icind  are,  undoubtedly,  not  very  rare  ;  Marshall  Hall,  Nase,  and 
J3ieger  hâve  mentioned  some.     I  also  hâve  seen  several. 

Thèse  cases  do  not,  however,  constitute  one  homogeneous  wliole, 
and  we  may  establish  at  least  two  principal  catégories.  In  the  first, 
we  hâve  a  true  motor  paralysis,  with  or  without  contracture, 
occupying  one  of  the  superior  extremities,  or  both  of  them  at 
the  same  time;  in  the  second,  we  hâve  no  paralysis,  properly 
so  called,  but  there  is  produced  in  the  upper  extremities,  on  inten- 
tional  movements,  a  motor  incoordination,  augmented  on  closure  of 
the  eyes,  and  comparable  in  evcry  respect  to  what  we  see  in  cases  of 
progressive  locomotor  ataxia.  In  thèse  patients,  also,  although  the 
primary  spinal  lésion  occupies  a  limited  point  of  the  dorsal  région 
of  the  cord,  we  observe,  in  addition  to  the  paralysis  of  the  lower 
extremities  which  this  détermines,  sometimes  ataxic  symptoms, 
sometimes  a  more  or  less  marked  paralysis  of  the  upper  extre- 
mities. 

What  is  the  reason  of  this  singular  complication  ?  In  order  to 
account  for  it,  Marshall  Hall,  as  I  hâve  said  above,  has  recourse  to 
a  sort  of  action  from  a  distance.  It  is  incontestable  that  certain 
expérimental  lésions  afFecting  the  inferior  segment  of  the  spinal 
cord,  may  react,  by  distant  action,  on  the  upper  régions  of  this 
nervous  column.  This  is  well  illustrated  by  an  experiment  of  M. 
Herzen,  referred  to  in  a  previous  lecture.  It  is  the  following  :  a 
fragment  of  caustic  potash  being  applied  on  the  lower  part  of  the 
cord  of  a  decapitated  frog,  it  is  impossible,  so  long  as  the  application 
lasts,  to  excite  reflex  movements  in  the  upper  extremities.     I  would 

^  "Rétrograde  Action  in  Spinal  Diseascs,"  'Dérangements  of  the  Nervous 
System,'  p.  248. 

2  "Mém.  sur  l'état  de  la  moelle  cpinicrc  dans  la  carie  vertébrale,"  iu  '  Mém. 
et  E,ccli.  Auat.  Patli.  sur  diverses  maladies.'     Paris,  1826. 


LATEEAL    FASOICULATED    SCLEROSIS.  2J 

xemind  you,  also,  that^  in  Lewisson^s  experiments,  acute  irritation 
of  the  viscéral  nerves  (rénal  and  utérine)  produced  temporary 
paralysis  in  the  lower  limbs.  Howcver  this  may  be^  it  seems  very 
difficult  to  explain,  by  an  action  of  this  kind^  permanent  symptoms 
like  ataxia,  or  paralysis  with  or  without  contracture  of  the  superior 
extremities  in  the  pathological  cases  which  engage  our  attention. 
According  to  my  judgmeut,  we  must  seek  the  solution  of  this 
problem  in  the  modifications  which  ascending  consécutive  sclerosis 
may,  under  certain  circumstances,  présent. 

Thus,  as  I  hâve  pointed  ont  to  you,  gentlemen,  ascending 
sclerosis,  consécutive  on  partial  lésions  of  the  dorsal  cord,  keeps 
strictly  limited,  in  the  posterior  columns,  to  the  médian  fascicles. 
Such,  at  least,  is  the  rule.  Xow,  as  was  demonstrated  in  our  last 
lecture,  a  lésion  of  the  médian  fascicles,  when  occupying  the  cer- 
vical enlargement,  has  not  the  effect  of  determining  the  appearance 
of  tabetic  symptoms  in  the  upper  extremities.  But,  when  once 
established,  consécutive  sclerosis  may  acquire  an  individual  exist- 
ence ;  and  it  may  happen  that,  spreading  beyond  the  limits  usually 
assigned  it,  it  may  in  certain  cases  invade  the  latéral  bands  of  the 
posterior  columns,  lésion  of  which,  as  you  know,  produces  inco- 
ordination. This  is  how  I  propose  that  you  should  interpret  the 
cases  of  the  second  category.  It  is  true  that  this  invasion  of  the 
external  bands  has  not  yet,  so  far  as  I  know,  been  ratified  by 
autopsy;  but  the  foregoing  considérations,  if  I  do  not  mistake, 
render  our  supposition  most  probable. 

The  facts  of  the  second  category  remain  to  be  reviewed.  The 
folio wing  is  the  explanation  I  w^ould  suggest  in  référence  to  them. 
Besides  the  fasciculated  sclerosis  of  the  posterior  médian  columns, 
in  cases  of  partial  lésion  of  the  dorsal  cord,  and  especially  when 
this  lésion  is  situated  very  high,  in  ,the  neighbourhood  of  the  cer- 
vical enlargement  for  instance,  there  nearly  always  exists  a  more  or 
less  well-marked  sclerosis  of  the  posterior  région  of  the  latéral 
columns.  This  ascending  latéral  sclerosis  remains  generally  in  a 
rudimentary  state,  and  then  does  not  détermine  any  symptom. 
But,  it  may,  in  certain  cases,  become  very  marked,  and  ascend 
to  the  bulbus,  principally,  I  repeat,  when  the  primary  partial  lésion 
occupies  the  superior  portion  of  the  dorsal  région.  Now,  paralysis 
of  the  upper  extremities,  sooner  or  later  followed  by  contracture, 
is  a  symptom  connected  with  latéral  fasciculated  sclerosis  occupying 
the  cervical  enlargement  of  the  spinal  cord. 


22  DESCRIPTION    OF    LOCOMOTOE    ATAXIA. 

To  sum  up  :  when  ascending  secondary  sclerosis  remains^  as  the 
rule  is,  limited  to  the  médian  fascicles  of  the  posterior  colurans, 
you  observe  in  the  upper  extremities  neither  paralysis,  nor  contrac- 
ture, nor  ataxic  movements.  If,  on  the  contrary,  the  external  bands 
are  invaded,  the  superior  extremities  will  be  attacked  by  motor 
incoordination.  Tinally,  the  paralysis  and  the  contracture  would 
appear  in  cases  where  the  consécutive  sclerosis  should  occupy,  to 
an  intense  degree,  the  latéral  columns  throughout  the  whole  height 
of  the  cervical  enlargement  of  the  spinal  cord. 

III. 

It  is  time,  gentlemen,  to  enter  upon  the  principal  object  of  this 
conférence.  We  hâve,  you  remember,  to  study  some  points  in  the 
clinical  history  of  progressive  locomotor  ataxia,  which  are  little 
known,  or,  at  ail  events  in  my  judgment,  insufficiently  known. 

If  the  cases  which  are  about  to  engage  our  attention  diverge  ail, 
though  in  différent  degrees,  from  the  common  type,  they  yet  ail 
are  related  to  it  by  some  essential  features  which  never  absolutely 
fail.  It  seems  to  me  useful,  before  entering  into  détails,  to  recall  in 
a  few  words  the  fundamental  characters  of  the  most  common  type. 
We  shall  be  the  better  able,  after  that,  knowing  the  analogies,  to 
set  out  and  define  the  contrasts, 

You  hâve  in  your  minds  the  description  of  locomotor  ataxia  as  it 
\ras  laid  down  by  M.  Ducheune  (de  Boulogne).  It  will  not  there- 
fore  be  necessary  to  enter  into  long  détails  to  refresh  your  memory 
concerning,  at  least,  its  principal  lines.  Ail  of  you  know  that 
progressive  locomotor  ataxia  is  a  primary  dironic  disease,  which 
attacks  différent  parts  of  the  nervous  System  simultaneously,  and 
•which,  as  a  gênerai  rule — there  are  some  qualifications  to  be  made 
on  this  point — as  it  advances  almost  inevitably  becomes  worse. 

The  spinal  lésion,  to  which  our  attention  has  chiefiy  been  given 
hitherto,  does  not  always,  of  itself  alone,  constitute  the  anatomo- 
pathological  ground-work.  To  it  are  referred  the  symptoms  termed 
spinal,  which,  undoubtedly,  form  one  of  the  most  salient  aspects  of 
the  symptomatic  picture  ;  but  it  is  rare  that  thèse  exist  separately. 
Usually,  there  is  superadded  a  whole  group  of  phenomena,  which 
are  generally  known  as  cephalic  symptoms,  and  whose  starting  point 
is  a  lésion  of  the  cérébral  or  bulbar  nerves,  such  as  the  optic  nerves, 
for  instance,  or  the  motor  nerves  of  the  eye. 

In  this  relation,  we  can  draw  a  parallel  between  progressive  loco- 


PEEIODS    OP   THE    DISEASE.  23 

motor  ataxia  and  the  disease  which  I  hâve  proposed  to  call  dis- 
seminated  sclerosis.  The  latter,  like  the  former^  invades  différent 
points  of  the  cerebro-spinal  System  simultaneouslj,  and  we  hâve 
cause  to  discriminate,  in  the  clinical  description  of  both  affections, 
between  (i°)  the  spinal  symptoms  and  {2°)  the  cephalic  symptoms. 
Disseminated  sclerosis,  like  locomotor  ataxia,  most  usually  pursues 
a  doomed  downward  path.  But  the  analogies  cease  there,  and,  in 
detailing  the  symptoms,  we  hâve  only  to  mark  différences  which 
almost  always  enable  us  to  make  a  diagnosis  without  difficulty. 

lY. 

It  is  customary  to  note  a  certain  number  of  periods  in  the  pro- 
gress  of  locomotor  ataxia,  which  is  supposed  to  evolve  in  a  normal 
manner.     Generally,  three  principal  periods  are  recognised. 

The  first  bas  received  the  name  oîprodromic  period.  Wherefore 
prodromic  ?  The  appellation,  perhaps,  is  ill  chosen,  for  when  the  first 
symptoms  appear  the  lésion  is  already  constituted  and  visible.  And, 
besides,  can  we  conceive  a  prodromic  period  capable  of  extending 
over  many  years — over  eight,  ten,  twelve,  tifteen  years,  for  instance  ? 
Perhaps  it  might  be  better  to  term  it  the  period  of  ligJdning  pains. 
Thèse  pains,  in  truth,  are  undoubtedly  one  of  the  most  tangible 
phenomena,  although  not  absolutely  constant.  However  this  be, 
the  disease,  in  this  period,  is  clinically  represented  by  two  orders  of 
symptoms  which  coexist  in  complète  cases,  and  show  themselves 
separately  in  imperfect  or  abortive  cases  {cas frustes)  as  they  are  also 
called.  Thèse  are  :  (1°)  The  cephalic  symptoms  (paralysis  of  a  motor 
nerve  with  ail  its  conséquences — more  or  less  marked  amblyopia)  ; 
(3°)  spinal  symptoms  represented,  at  this  epoch,  by  the  ligJdning 
pains. 

In  the  second  period,  termed  period  of  establisJied  disease,  which 
might  also  be  named  period  qf  motor  incoordination,  the  clinical 
picture  is  composed,  in  the  first  place,  of  the  symptoms  of  the  first 
period,  in  varions  proportions  ;  thus  the  cephalic  symptoms  persist 
and  show  themselves  in  a  more  aggravated  and  marked  form  ;  the 
lightning  pains  occasionally  become  more  intense.  But  the  capital 
point  is  this:  the  spinal  symptoms  hâve  undergone  an  important 
modification. 

We  observe,  in  the  first  place,  tabetic  incoordination  of  the 
voluntary  movements.  In  what  does  this  symptom  consist  ?  You 
know   that,   whilst  the  inferior  extremities   hâve  preserved  their 


24  PEEIOD    OF    LIGHTNING    PAINS. 

dynamometric  power,  there  is  asynergia — tliat  is  to  say,  a  vicious. 
and  inappropriate  association  of  the  elementary  motor  acts,  an 
asynergia  whose  effect  is  to  produce  disturbance  in  the  movements 
of  the  patient  whilst  walking,  and  in  his  position  whilst  standing,, 
and  even  to  render  botli  impossible. 

Simultaneously,  a  little  later  or  a  little  sooner^  we  find  also. 
différent  dérangements  in  the  transmission  of  sensory  impressions, 
exhibited  :  (i°)  by  abolition  of  the  différent  modes  of  cutaneous  sen- 
sibility  ;  (2°)  by  insensibility  of  the  more  deep-lying  parts,  such  as, 
the  muscles,  articulations,  bones,  &c. 

The  third  period  would,  perhaps,  deserve  to  be  qualified  as  the 
2)aralytic period.  Whilst  the  other  symptoms  become  aggravated, 
and  the  upper  extremities,  until  now  free,  are  being  invaded  m 
their  turn,  after  the  manner  indicated  in  our  anatomical  préface^ 
we  remark  at  the  same  time  that  the  lower  extpemities  become 
affected  by  real  paralytic  debility,  which  gradually  replaces  the  in- 
coordination. Then,  also  simultaneously,  nutrition  frequently 
suffers,  in  a  gênerai  manner  ;  the  patients  grow  thin,  and  it  is  very 
common  to  find  symptoms  of  phthisis  exhibited.  In  other  cases, 
nutrition  is  slowly  and  locally  affected;  the  muscles  waste  away  in 
the  limbs  deprived  of  motion  ;  a  tendency  to  the  production  of 
sacral  eschars  is  shown  ;  and  symptoms  of  ulcerous  cystitis  appear, 

Such,  in  summary,  gentlemen,  is  the  common  type  of  progressive 
locomotor  ataxy.  We  are  now  in  a  position  to  set  ont  the  anoma- 
lies, the  déviations  from  this  standard. 

V. 

Let  us  dévote  our  attention,  in  the  first  place,  to  the  study  of 
the  first  period.  That  is  the  time  when  it  is  most  important  ta 
recognise  the  existence  of  the  disease  ;  for,  as  it  is  then  only  begin- 
ningjWe  should  hope  that  it  would  be  the  less  difficult  to  arrest  its 
course.  Nevertheless,  at  this  period,  it  is  very  frequently  mis- 
understood.  Many  persons  also  imagine  that  the  disease  only 
begins  at  the  time  when  the  incoordination,  the  ataxia  from  which 
it  gets  its  name,  has  become  manifest.  Now,  at  that  period,  I 
repeat,  the  disease  may  hâve  been  in  existence  for  ten,  fifteen,  or 
even  twenty  years.  Besides,  it  sometimes  stops  of  itself  at  this 
period,  without  ever  advancing  farther,  but  also  without  retrograd- 
ing.  Hence  it  is,  gentlemen,  that  we  shall  endeavour  to  show  you 
that  the  liglitning  pains  and  the  cephalic  symptoms  may  présent 


BOEING    AND    LANOINATING   PAINS.  25- 

themselves — and  do  in  reality  présent  themselves — generally  with 
almost  spécifie  characters,  which,  in  tlie  absence  of  ail  other  con- 
comitant phenomena,  commonly  allow  us  to  recognise  the  disease 
for  what  it  is,  and  to  designate  it. 

Let  us  pause,  first,  at  the  lightning  pains,  reserving  for  a  near 
opportunity,  a  thorough  examination  of  the  cephalic  symptoms.  To- 
show  you  the  importance  of  this  question,  it  is  enough  to  remiud 
you  that,  during  long  years,  thèse  pains  may,  of  themselves  alone^ 
compose  the  entire  symptomatology  of  the  disease. 

Lightning  pains,  besides,,  are  found  in  a  very  large  majority  of 
the  cases  of  progressive  locomotor  ataxia.  It  caunot  be  deaied,  how- 
ever,  that  they  are  absent  from  some  patients  ;  but  that  is  a  very 
exceptional  occurrence.  Thus,  according  to  the  statistical  table  of 
M.  Cyon_,  which  includes  203  cases,  lightning  pains  were  expressly 
mentioned  in  138;  in  8  cases  only  was  their  absence  remarked. 

The  varieties  which  they  présent  induce  us  to  form  two  caté- 
gories : 

1°.  Boring  pains. — Pains  of  this  first  variety  are  compared  by 
the  patients  to  those  which  would  be  caused  by  the  abrupt  and 
sudden  introduction  of  a  pointed  instrument,  an  awl  or  a  poignard, 
if  this  were  twisted  round  on  being  pressed  into  the  flesh.  Thèse 
pains  are  limited  to  a  point,  and  are  commonly  found  in  the  vicinity 
of  an  articulation.  However,  it  would  be  going  too  far  to  assert 
that  they  always  spare  the  body  of  the  limbs.  Generally,  during 
a  paroxysm,  the  boring  pains  alternate  in  différent  places  and 
occupy  successively  several  points  on  both  sides  of  the  body. 

At  the  point  they  occupy,  a  more  or  less  well-marked  hyperses- 
thesia  is  momentarily  produced,  within  a  very  circumscribed 
area  ;  the  least  rub  exaspérâtes  the  pain,  whilst  a  somewhat  strong 
pressure  gives  relief.  The  inferior  extremities  are  always  affected, 
by  préférence  ;  nevertheless,  the  superior  extremities,  the  arms,. 
head,  and  body  are  far  from  being  always  respected. 

2°.  Lancinating  pains. — Pains  of  the  second  variety  which, 
indeed,  generally  coexists  with  the  first,  deserve,  properly  speaking, 
the  name  of  lightning,  or  fulgurant  pains.  They  seem  to  folio w 
the  course  of  a  nerve  along  which  they  shoot,  like  a  flash  of 
lightning.  The  conséquence  is  that  an  extremity,  or  an  area  of 
varying  extent  is  rapidly  traversed  by  the  painful  fulguration.  On 
account  of  the  short  continuance  of  the  distressing  sensation,  it  is 
often  difficult  to  define  exactly  the  affected  nerve.     This,  however. 


26  CONSTEICTIVE    PAINS. 

may  be  very  distinctly  done,  though  the  occasion  is  rare,  wlien 
cutaneous  éruptions  break  ont  along  the  course  of  the  nerve  which 
is  the  seat  of  the  pain.  Such  an  occurrence  marked  the  case  of  a 
patient,  named  Magdaliat,  whom  you  now  see,  and  who,  during  a 
most  intense  paroxysm  of  pain,  exhibited,  in  succession,  éruptions 
of  ecthyma  over  the  course  of  the  lesser  sciatic  nerve  at  first, 
then  over  that  of  the  internai  saphenous  nerve.  Even  now,  you 
can  still  see  the  scars  resulting  from  those  lésions,  which  affected 
him  four  or  five  years  ago. 

3°.  Constrictive  pains. — Besides  the  lightning  pains,  we  hâve 
also,  in  ataxia,  to  take  the  constrictive  ^^ains  into  considération  : 
thèse  pains  super vene  either  at  the  same  time  as  the  preceding,  or 
irrespective  of  them.  Their  duration  is  longer,  and  their  persistance 
greater.  The  patients  feel  as  if,  in  certain  places,  the  extremity 
were  seized  in  a  vice.  Does  the  pain  affect  the  trunk?  They 
compare  it  to  the  constriction  caused  by  an  over-tight  cuirass,  or  a 
corset  too  closely  laced.  The  constrictive  pains  become  frequently 
exasperated  during  the  prédominance  of  boring  or  fulgurant  crises, 
properly  so  called.  We  may  résume  in  a  few  words  the  charac- 
teristics  of  fulgurant  pains  : 

1°.  The  painful  fulguration  (or  lightning  pain)  has  but  a  tran- 
sient  duration,  as  its  name  indicates. 

2°.  It  is  repeated  at  varying  intervais,  so  as  to  constitute 
paroxysms  which  last  for  four,  five,  or  eight  days. 

3°.  The  pain  attains  its  maximum  of  intensity  chiefly  during 
the  night. 

4°.  The  remissions  which  separate  the  paroxysms  may  be  per- 
fectly  free  ;  the  constrictive  pains  alone  form  exceptions  to  the  rule, 
and  persist  to  a  certain  degree  during  the  intervais  of  the  crises. 

5°.  The  return  of  the  paroxysm  varies  greatly  :  it  happens 
every  fortnight,  every  month,  every  second  or  third  month,  some- 
times  after  still  longer  intervais. 

6°.  Sometimes  the  shootiug  pains  are  of  moderate  intensity, 
and  it  is  requisite  to  specially  question  the  patient  about  them  to 
revive  their  memory  ;  again,  on  the  contrary,  they  are  of  extrême 
violence  and  compel  the  unfortunate  sufferer  to  give  utterance  to 
frightful  shrieks.  In  this  hospital,  where  the  number  of  such 
patients  is  large,  we  hâve  often  to  witness  scènes  of  this  kind. 


SYMPTOMATIC    LIGHTNING  PAINS.  27 

VI. 

The  numerous  facts  whicli  I  liave  had  occasion  to  observe  lead 
me  to  admit  tliat  lightning  pains,  when  manifested  in  the  manner  I 
hâve  tried  to  depict,  are  truly  characteristic,  I  will  not  say  of 
locomotor  ataxia,  but  rather  of  posterior  riband-sclerosis,  in  so  far 
at  least  as  that  the  lésion  bas  invaded  the  intra-spinal  course  of  the 
internai radicidar fascïcles  (external  bauds  of  the  posterior  fascicles). 
This  réserve  was  necessary.  You  bave,  doubtless,  not  forgotten 
that  the  médian  fascicles  (Goll's  column)  may  be  subject  to  sclerosis, 
without  shooting  pains  supervening;  and,  on  the  other  hand,  that 
thèse  pains  exist  when  the  scierons  lésion  bas  remaiaed  limited  to 
the  course  of  the  radicular  fascicles.  Sclerosis  of  the  external 
ribands  would,  alone,  as  you  perceive,  constitute  the  anatomical 
substrakim  of  the  lightning  pains. 

Knowing  this  you  will  not,  therefore,  gentlemen,  be  surprised  to 
find  lightning  pains  figuring,  now  and  again,  in  the  symptomato- 
logy  of  différent  diseases,  other  than  ataxia,  in  which  the  posterior 
fascicles  may  be  invaded  accidentally,  as  it  were,  by  scierons 
inflammation.  Such,  for  instance,  is  the  case  as  regards  dissemi- 
nated  sclerosis.  It  is  not  rare,  in  this  affection,  to  find  différent 
tabetic  symptoms,  and,  particularly,  shooting  pains  superadded  to 
the  proper  symptoms  of  the  disease.  In  such  circumstances,  I 
hâve  many  times  noted  that  the  sclerous  nodules  had  invaded  the 
posterior  columns_,  which  they  occupied  throughout  a  large  extent, 
both  transversely  and  vertically. 

Paroxysms  of  lightning  pains  are  also,  somewhat  frequently, 
observed  in  progressive  gênerai  paralysis  ;  they  should  certainly 
be  referred,  in  this  case,  to  altérations  of  the  posterior  fascicles 
which,  as  shown  by  Drs.  Magnan  and  Westphal,  are  a  common 
accompaniment  of  the  ordinary  lésions  of  gênerai  paralysis. 

I  was  consulted  a  few  years  ago  by  two  patients  who  mentioned 
a  host  of  odd  nervous  symptoms  which  I  believed  might  be 
referred  to  hypochondria.  Thèse  two  patients  complained  more 
particularly  of  pains,  returning  in  paroxysms,  which  were  entirely 
similar  to  those  of  locomotor  ataxia.  Ultimately,  both  of  them 
exhibited  the  symptoms  of  progressive  gênerai  paralysis.  I  bave 
no  doubt  that,  in  their  case,  the  external  bands  of  the  posterior 
fascicles  had  been  already  affected  at  the  time  when  lightning  pains, 
almost  alone,  constituted  the  whole  of  their  disease.     Some  of  the 


28  VISCERAL   SYMPTOMS. 

symptoms  mentioned  in  the  clinical  account  of  chronic  alcoholism 
recall  the  description  of  lightning  pains  ;  thus  Magnus  Huss  has 
marie  spécial  mention  of  the  lancinatïng  pains,  occasionally  very 
severe,  of  which  alcoholic  patients  complain. 

Recently^  Mr.  Wilks  and  Dr.  liockhart  Clarke^  hâve  called  atten- 
tion to  a  form  of  paraplegia  which,  it  appears,  is  rather  frequently 
observed  in  London  amongst  women — and  even  amongst  ladies — 
and  which  they  unanimously  designate  alcoholic  paraplegia.  One 
of  the  most  salient  features  of  this  pathologie  form  appears  to  be 
the  existence  of  pains,  recurring  in  paroxysms,  and  which  the 
patients  compare  to  electric  shocks.  The  pains  exist  alone  for  a 
long  time  before  motor  disorders  arrive  in  addition.  We  hâve 
reason  to  ask  ourselves  if,  hère  also,  ve  bave  not  to  deal  with  a 
particular  form  of  tabès,  of  alcoholic  origîn,  indeed,  but  always 
referable  to  some  lésion  of  the  posterior  columns,  which  morbid' 
anatomy  will,  doubtless,  one  day  discover. 

I  hâve  sometimes  observed  lightning  pains,  comparable  to  those 
of  ataxia,  in  partial  myelitis,  and  in  Pott's  disease.  In  several  of  thèse 
cases,  at  the  autopsy,  I  identified  an  extensive  fasciculated  lésion  of 
the  posterior  columns,  which  would  account  for  the  présence  of 
spécial  pains. 

Apart  from  the  cases  which  hâve  just  been  reviewed,  and 
whose  dismissal  can  always  be  made  with  ease,  by  taking  note 
of  the  concomitant  symptoms,  the  lightning  pains  may  be  referred, 
almost  with  certainty,  to  the  particular  form  of  posterior  sclerosis 
which  leads  to  progressive  locomotor  ataxia.  There  do,  indeed, 
occur,  now  and  again,  in  practice,  some  difificulties  of  appréciation 
to  which  I  will  ask  your  attention  ;  but,  in  reality,  thèse  are  some- 
what  rare.  Besides,  the  situation  is  very  often  simphfied  by  the 
adjunction  to  the  shooting  pains  of  certain  symptoms  which  hâve, 
like  them,  a  spécial  character.  Such,  for  instance,  are  the  sym- 
ptoms of  the  ataxia,  known  as  cephalic,  which  we  shall  hâve  soon 
to  discuss;  such  also  are  other  symptoms,  less  noticed  though 
often  fréquent  enough,  which  may  be  denominated  viscéral  symptoms, 
because  they  clearly  attest  the  participation  of  the  viscéral  nerves 
of  the  thorax  and  abdomen. 

In  this  group  of  viscéral  symptoms,  I  will  first  of  ail  point  out 
the  vesical  and  urethral  j)ai7is,  which  sometimes  show  themselves  at 
the  time  when  the  lightning  pains  hold  dominion,  and  which  are 

'  Lancet,  1872. 


GASTRIO    CEISES.  29 

accompanied  by  a  fréquent  désire  to  micturate,  tlie  act  being  also 
the  occasion  of  acute  pains  in  tlie  canal. 

In  the  second  place,  I  would  refer  to  pains  of  a  peculiar 
cbaracter,  the  seat  of  which  is  in  the  rectum,  and  which  supervene 
in  the  same  circumstances  as  the  vesical  pains.  In  the  case  of  one 
patient,  M.  C,  who  sufFered  from  thèse  rectal  pains,  in  their  most 
developed  type,  they  had  preceded,  by  seven  or  eight  months,  the 
manifestation  of  the  shooting  pains,  which  they  afterwards  accom- 
panied. They  came  on  suddenly,  and  were  marked  by  a  sensation 
similar  to  what  might  be  caused  by  the  abrupt  and  coercive 
entrance  of  a  voluminous  body  iuto  the  rectum,  ïhis  is  how  the 
patient  described  them,  and  he  added  that,  at  the  close  of  the 
paroxysm,  there  supervened  a  pressing  need  of  expulsion,  and  even 
occasionally  an  actual  expulsion  of  fsecal  matter.  Thèse  pheno- 
mena  were  usually  reproduced  twice  or  thrice  a  month  ;  habitually, 
there  was  superadded  a  pressing  désire  to  micturate,  with  pains 
during  émission.  During  several  months  catheterism  and  rectal 
examination  bad  been  frequently  recurred  to,  the  médical  attendant 
never  suspecting  the  nature  of  the  disease.  Not  until  long  after 
did  the  apparition  of  shooting  pains  arrive  to  define  the  situation. 

VII. 

But  of  ail  the  viscéral  symptoms  which  may  display  them- 
selves,  from  the  period  of  lïghtning  pains,  one  which  is  at  once 
the  most  remarkable  and  the  least  known,  if  I  mistake  not,  is  that 
which  I  hâve  proposed  to  designate  by  the  name  of  gastric  crises. 

Thèse  gastric  or  gastralgie  crises,  as  you  may  prefer  to  call  them, 
ofFer  truly  spécial  characters.  Very  often,  however,  their  real  sig- 
nification remaining  misunderstood,  they  are  the  occasion  of  grave 
errors  of  diagnosis. 

This  is  not  a  rare  symptom,  nor  has  it  been  complctely 
passed  over.  Mention  of  it  will  be  found  iu  a  considér- 
able number  of  observations  collected  by  différent  authors,  and 
particularly  in  case  No.  176  of  M.  Topinard's  excellent  work. 
But  the  connection  which  really  exists  between  gastric  crises  and 
locomotor  ataxia  appears  to  me  to  hâve  been  pointed  out,  for  the 
iirst  time,  by  M.  Delamarrc,  author  of  a  thesis  bearing  date  iH66.^ 
In  1868,  in  my  lectures,  I  took  care  to  insist  upon  the  import- 
ance which  I  attached  to  this  symptom,  and  M.  P.  Dubois,  one  of 
my  auditors,  consigned  in  his  inaugural  dissertation  of  the  same 
'  "  Des  troubles  gastriques  dans  l'ataxie  locomotrice." 


30  GASTRIC   CEISES  :    TIIEIR    CHARACTERS. 

year  the  resuit  of  tlie  studies  whicli  lie  had  madein  référence  to  this 
subject,^  in  concert  witli  M.  Bourneville. 

You  should,  also,  be  informed  that,  in  1858,  Dr.  Gull,  in  the 
valuable  collection  entitled  "  Cases  of  Paraplegia/^  whicli  he  pub- 
lislied  in  '  Gxxy's  Hospital  Eeports/  pointed  ont  the  relation  which 
seemed  to  him  to  exist  between  certain  gastric  symptoms  and  an 
affection  of  the  cord_,  which  evidently  resembles  progressive  loco- 
motor  ataxia_,  as  at  présent  described. 

We  shall,  aiso,  meet  again  with  gastric  symptoms  closely  ana- 
logouSj  at  least,  with  those  for  which  I  wish  to  obtain  your  atten- 
tion, in  other  spinal  diseases  than  posterior  sclerosis,  for  instance 
in  spinal  gênerai  par  aly  sis.  The  latter  affection,  when  it  prédomi- 
nâtes in  the  upper  extremities,  reminds  us,  by  some  of  its  characters, 
of  saturnine  paraplegia,  and  the  cardialgic  or  enteralgic  crises  which 
accompany  it  are  then  sometimes,  but  very  wrongly,  regarded  as 
lead  colics.  There  is  a  difBculty  of  diagnosis,  concerning  which  I 
sliall  hereafter  hâve  to  enter  into  further  détails. 

But  it  is  time  to  tell  you  in  what  thèse  gastric  crises  consist. 
Suddenly,  and  generally  at  a  period  when  a  paroxysm  of  shooting 
pains  has  seized  upon  the  extremities,  the  patients  complain  of  pains 
which,  starting  from  the  groins,  seem  to  ascend  both  sides  of  the 
abdomen,  and  to  iix  themselves  in  the  epigastric  région.  At  the 
same  time,  they  complain  of  pains  situated  between  the  shoulders, 
which  radiate  around  the  base  of  the  trunk  in  a  lightuing-like 
manner.  Then  the  pulsation  of  the  heart  commonly  becomes 
violent  aod  precipitated.  Professor  Rosenthal,  who  has  occasion- 
ally  witnessed  thèse  paroxysms,  mentions  a  case  in  which  the 
puise  was  slower  than  usual  during  the  attack.  As  regards  my 
own  expérience,  I  bave  always  observed,  on  the  contrary,  in  such 
cases,  a  marked  accélération  of  the  puise,  which  is  accompanied^by 
no  élévation  of  the  central  température. 

Frequency  of  the  puise  without  fever  is,  in  truth,  a  very  common 
incident,  from  the  first  periods  of  the  ataxia,  and  even  apart  from 
the  gastric  crises  and  fulgurant  paroxysms,  at  a  time  when  no  trace 
of  raotor  incoordination  as  yet  exists. 

Almost  incessant  and  extremely  distressing  vomiting"is  often 
associated  with  the  gastric  crises.  Food  is  first  ejected,  and  then  a 
mucous  colourless  liquid,  sometimes  mixed  with   bUe  or  tinged 

*  "  Étude  sur  quelques  points  de  l'histoire  de  l'ataxie  locomotrice."  '  Thèses 
de  Paris,'  1868. 


GASTEIC    CEISES  :    THEIR    CHARACTEES.  31 

with  blood.  An  intense  feeling  of  sickness  and  vertigoes  are 
superadded  to  the  vomiting  and  cardialgic  pains  ;  thèse  may  be 
really  excruciating,  and  the  situation  is  then  the  more  afïlictiûg 
because  the  painful  fulgurations  often  at  the  same  time  affect  the 
extremities  with  an  exceptional  intensity  of  torture. 

The  gastric  crises  of  ataxic  patients  habitually  persist,  like  the 
fulgurant  crises,  almost  without  respite  for  two  or  three  days  ;  and 
it  is  very  remarkable  tliat,  in  the  intervais  of  thèse  paroxysms,  the 
functions  of  the  stomach  are  generally  performed  with  great 
regularity.  Such  crises  may  appear  at  the  beginning  of  the  dis- 
ease,  and  may  for  long  years  form,  together  with  shooting  pains, 
the  whole  symptomatology  of  the  disease.  When  the  ataxia  is  fuUy 
established,  and  motor  incoordination  has  been  developed,  the 
gastric  crises  do  not  therefore  always  disappear  ;  often,  on  the  con- 
trary,  they  are  reproduced  with  every  paroxysm  of  shooting  pain 
until  the  fatal  termination.  Such  was  the  case  with  the  patient 
Ménil,  to  mention  but  one,  whom  we  hâve  had  full  opportunity  of 
observing  during  her  sojourn  of  over  six  years  in  thèse  wards. 

That  is,  undoubtedly,  a  form  of  cardialgia  which  is  verv  singular, 
very  remarkable  in  ail  its  bearings.  Nevertheless,  you  will  not 
find  it  mentioned,  at  least  I  believe  it  is  not  mentioned,  in  any  of 
even  the  most  récent  spécial  treatises  on  diseases  of  the  stomach. 

Many  a  time  I  hâve  seen  this  symptom  diverting  the  attention  of 
the  physician,  and  causing  him  to  misapprehend  the  real  nature  of 
the  disorder  ;  I  also  hâve  several  times  fallen  into  the  snare  in 
other  days.  A  notary  came  from  the  provinces,  ten  years  ago,  to 
consult  me  concerning  attacks  of  cardialgia,  presenting  the  characters 
which  I  hâve  just  described  ;  he  sufïered  likewise  in  the  extremities 
from  paroxysmal  pains  which,  however,  were  not  very  acute.  I 
was  not  then  aware  of  the  link  which  unités  thèse  différent  phe- 
nomena.  The  gastric  crises  hâve  disappeared,  but  the  patient 
suffers  to-day  from  ail  the  symptoms  of  locomotor  ataxia  of  the 
most  characteristic  kind. 

The  first  time  it  was  given  to  me  to  recognise  the  true  signi- 
fication of  gastric  crises,  occurred  when  attending  a  young  physician, 
who,  besides  thèse  crises,  suffered  from  shooting  pains  and  hydar- 
throsis  of  one  of  the  knees,  spontaneously  developed  (arthropathy 
of  ataxic  patients).  Motor  incoordination  did  not  show  itself,  in 
his  case,  tiïl  some  months  later.  The  whole  of  this  group  of 
symptoms — gastric    crises,   shooting   pains,    and    arthropathies — 


-32  ILLUSTRATIVE   CASES. 

which  liave  no  affinity  in  appearance,  becomes  invested  with  an 
almost  spécifie  character  when  looked  upon  in  a  true  liglit. 

I  hâve  also  seen  gastric  crises  coexist  witli  lightning  pains, 
during  more  than  five  years,  without  being  accompanied  by  motor 
disorders,  in  the  case  of  M.  T.  The  diagnosis  was  rendered 
easy  in  this  case,  owing  to  the  existence  of  incipient  atrophy  of 
one  of  the  optic  nerves.  The  opinion  which  I  expressed  almost 
from  the  first,  concerning  the  nature  of  the  case,  was,  nevertheless, 
keenly  contested  by  several  physicians  who  visited  the  patient.  To- 
day my  -anticipations  hâve  been  found  only  too  amply  justified. 

In  treating  this  subject  of  gastric  crises,  we  are  not  compelled  to 
recur  merely  to  memories.  I  can,  in  fact,  introduce  to  you  a 
certain  number  of  patients  in  whom  you  eau  study  this  phenomenon. 
At  the  same  time,  this  will  furnisli  you  with  an  opportunity  for 
observing  abortive  ataxia,  in  some  of  the  varions  forms  which  it 
may  assume, 

1°.  The  patient  Mar — ,  at  présent  aged  46  years,  suffered  during 
a  dozen  years  from  hghtning  pains,  recurring  in  paroxysms,  which 
often  appear  in  eombination  with  gastric  crises.  The  latter  occur 
about  once  every  three  or  four  months  ;  they  are  usually  of  extrême 
intensity,  and  when  the  patient  is  a  prey  to  thèse  pains,  she  shrieks 
aloud,  contorts  herself,  and  assumes  the  most  extraordinary  attitudes. 
At  the  close  of  a  few  days  the  attack  terminâtes  suddenly,  as  if  by 
enchantment.  Digestion  is  regular,  in  the  intervais.  The  diagnosis  is 
facilitated  in  this  case  by  the  existence  of  strabismus,  dating  from 
the  time  when  the  lightning  pains  first  began  to  appear.  There  is 
no  incoordination  in  the  movements  of  the  upper  or  lower  extremi- 
ties.  The  gait  is  regular,  and  the  patient  can  stand  a  long  time 
without  fatigue.  However,  when  she  closes  her  eyes  it  becomes 
somewhat  more  difiicult  for  her  to  stand  or  to  walk. 

3°.  Coud — ,  aged  55.  Twenty-nine  years  ago  this  woman 
•became  blind.  The  lésion  of  the  fundus  oculi  consists  of  pearly 
atrophy  of  the  papilla3.  She  bas  been  subject  to  lightning  pains 
during  ten  years.  They  often  occupy,  not  only  différent  parts  of 
the  extremities,  but  also  the  occipital  région  and  the  nape  of 
the  neck.  Cardialgic  crises  frequently  coexist  with  fulgurant 
paroxysms,  and  are  accompanied  by  vomiting.  This  somewhat 
exceptional  seat  of  lightning  pains,  in  the  occiput  and  nape  of 
neck,  deserves  to  be  remarked  on  account  of  the  vomiting, 
which  often  appears  at  the  same  time  as  they  do.     Such  a  con- 


CASES   OP    GASTRIC  CRISES.  33 

currence  of  circumstances  might  render  the  diagnosis  obscure,  and 
lead  one  to  think  of  the  existence  of  a  cérébral  or  cerebellar  lésion. 
Tins,  however,  is  a  point  to  which  we  shall  return.  Hitherto^, 
■Coud —  lias  not  suffered  from  any  disorder  of  locomotion. 

3°.  The  patient  Deg — ,  aged  52  years,  has  been  blind  fifteen 
years.  Hère,  also,  pearly  atrophy  of  the  optic  nerves  has  been 
found.  The  attacks  of  lightning  pains,  which  occupy  the  most 
diverse  parts  of  the  body,  and  frequently  also  the  brow,  the  occiput, 
the  nape  of  the  neck,  commenced  to  appear  at  the  time  when  the 
blindness  began.  They  are  often  accompanied  by  gastric  crises  of 
great  intensity.  The  functions  of  the  stomach,  in  the  intervais  of 
the  attacks,  are  regular.  No  symptom  of  motor  incoordination 
exists  in  her  case. 

4°.  Audib — ,  aged  about  ^^,  suffers,  now  and  again,  from 
gastric  crises  of  a  thoroughly  excruciating  kind,  accompanied  by 
incessant  vomiting,  generally  coming  on  at  the  same  time  as  the 
attacks  of  shooting  pains  which  affect  her  lower  extremities. 
Thèse  phenomena  hâve  been  in  existence  for  five  or  six  years.  Two 
years  ago,  spontaneous  luxation  of  the  right  hip  supervened,  fol- 
lowed,  some  months  after,  by  luxation  of  the  left  hip  (ataxic  arthro- 
pathy).  Some  symptoms  of  motor  incoordination  hâve  shown 
themselves  within  the  past  twelve  months. 

I  shall  not  pursue  the  subject  further  for  the  présent. 


VOL.   II. 


LECTUEE  III. 
TABETIC  AMAUROSIS. 

ScJMMARY. — Cephalic  symptoms  in  locomoior  ataxia.     Lésions   of 
ihe  cranial  and  bulbar  nerves.    Progressive  grey  hiduration  of 
the  opt'ic  nerve.     Progressive  atropliy  of  the  papilla.     Neces- 
sity  of  ophthalmoscojjic  examination  in  the  diagnosis  of  sortie 
cérébral  affections. 

Isolated  existence  of  tabetic  amaurosis  :  its  frequency. 
C/mracters  of  tlie  anatomic  lésion  of  the  optic  nerve,  seen  by 
the  nahed  eye  and  nnder  the  microscope.  Appearance  of 
papilla  :  i°,  in  the  normal  stale  ;  'f ,  in  progressive  grey  indura- 
tion. Functional  dérangements  accompanying  grey  induration 
of  the  optic  nerve  and  papilla.  Modifications  of  the  papilla 
in  cases  of  embolism  of  the  arieria  centralis  of  the  retina,  in 
glycosuria,  syphilitic  retino-choroiditis,  and  gênerai  par aly sis. 

Neuro-retinitis  :  its  for  ms  and  symptoms  ;  différences  which 
distinguish  it  from  tabetic  amaurosis.  Clinical  facts  démon- 
strating  the  importance  of  the  signs  furnished  by  the  ophthal- 
moscope. 

Gentlemen, — It  is  my  intention  to  discuss  to-day,  in  your 
présence,  some  points  connected  with  those  symptoms  of  ataxia 
which  we  hâve  denominated  cephalic  symptoms.  Thèse  symptoms 
correspond  to  varions  lésions  of  the  cranial  and  bulbar  nerves.  From 
the  early  periods,  as  we  hâve  already  said,  the  lightning  pains 
(which  are  then  the  sole  représentatives  of  the  spinal  lésion)  gene- 
rally  appear  combined,  in  différent  proportions,  with  disorders  of 
the  bulbar  and  optic  nerves.  I  will  add  that  the  latter  may,  in  the 
évolution  of  the  morbid  process,  occasionally  précède  those  very 
lightning  pains,  and  thus  show  themselves  completely  isolated 
during  many  months,  nay  even  during  many  years.  This  shows 
what  clinical  interest  attaches  to  their  study. 


VISUAL    DISOEDEES.  35 

There  is,  perhapS;,  iiot  one  of  the  bulbar  nerves  wliich  may  nofc 
be  affected  at  this  same  epoch  ;  but,  commonly,  tbe  first  invaded 
are  the  oculo-motor  nerves.  Taken  in  order  of  frequency  we  bave, 
first,  the  nerves  of  tlie  third  and  of  the  sixth  pairs  ;  then,  but  far 
bebind,  cornes  the  pathetic  nerve;  the  facial,  the  hypoglossus,  and 
the  fifth  pair  do  not  themselves  always  escape.  The  symptoms 
which  are  correspondent  to  thèse  lésions  are  shown  in  some  patients 
by  a  paralytic  state,  in  others  by  pains.  But,  of  ail  the  cranial 
nerves,  the  optic  nerves  are  those  which,  in  this  disease,  deserve  to 
be  specially  considered,  on  account  of  the  great  frequency  of  their 
altération,  and  of  the  gravity  of  the  affection  which  this  lésion 
détermines.  Hence,  in  référence  to  this  subject,  we  shall  enter  into 
some  détails. 


The  visual  disorders  of  ataxia  may  be  classed  under  two  principal 
heads.  Thèse  are:  i°,  disorders  of  accommodation;,  diplopia,  con- 
nected  with  more  or  less  transitory  and  fugitive  lésions  ;  2°,  visual 
disorders  depending  on  a  peculiar  lésion  of  the  optic  nerve.  Thèse 
are  far  more  serious  than  the  former,  for  the  diminution  of  sight^ 
like  the  lésion  which  produces  it,  is  distinguished  by  a  progrcss 
which  seems  almost  inevitably  destined  to  increase  and  encroach. 

The  lésion  of  the  optic  nerves  in  question  might  be  termed 
progi-essive  grey  induration.  This  would  distinguish  it,  on  the  one 
hand,  from  the  lésion  of  thèse  same  nerves  in  disseminated  sclerosis, 
which  runs  a  very  analogous  course,  the  progress  of  which  is,  so  to 
speak,  less  inévitable;  and,  on  the  other  hand,  from  the  lésions 
known  under  the  name  of  optic  neuritis.  In  the  last  two  cases,  in 
spite  of  some  features  of  resemblance,  the  lésions  and  the  symptoms 
differ  in  an  essential  manner  from  what  they  are  in  tabetic  optic 
atrophy. 

During  life,  progressive  grey  induration  of  the  optic  nerves  is 
identified  by  spécial  ophthalmoscopiô  characters  which  answer  to 
what,  in  ophthalmology,  is  known  as  progressive  atrophy  of  the 
papilla.  Thèse  characters  are,  according  to  certain  authors, 
almost  spécifie  ;  and,  even  apart  from  the  accompanying  functional 
disorders,  which  also  ofFer  pcculiarities  of  great  clinical  interest, 
are  such  as  to  enable  us  to  diagnosè  the  ataxia,  or  sclerosis  of 
the  posterior  columns,  if  it  already  exists,  oxioforesee  itsadvent,  at 
a  more  or  less  early  date  if  it  be  not  yet  distinctly  established.     The 


36  TABETIC    AMATJEOSIS. 

assertions  of  MM.  Jaeger,  Wecker,  and  Galezowski,  are  précise 
upon  this  subject. 

We  will  see,  gentlemen,  wliat  judgment  we  should  form  in 
référence  to  it,  I  should,  however,  say  beforehand  that,  having 
frequently  witnessed  the  certainty,  the  exactness  of  the  diagnosis 
laid  down  by  the  gentlemen  versed  in  thèse  matters,  I  hâve  been 
induced  to  share,  to  a  large  extent  at  least,  in  the  conviction  which 
they  hold.  Although  thèse  facts  are  not  absolutely  within  my 
spécial  sphère,  I  shall,  however,  request  your  permission  to  enter 
into  some  détails  respecting  them. 

My  undcrtaking  will,  I  trust,  find  its  justification  in  the  interest 
which  should  attach  to  ail  questions  that  concern  the  diagnosis  of 
tabès  dorsalis. 

IL 

A  summary  statement  of  two  points,  relating  to  the  difticulties 
mentioned,  will  suffice  to  illustrate  how  important  it  is  for  us,  as 
physicians,  to  familiarise  ourselves  as  much  as  possible  with  the 
regular  examination  of  the  fundus  of  the  eye. 

In  the  first  place,  I  shall  endeavour  to  demonstrate  to  you  that 
ataxia  may  présent  itself  surrounded  by  symptoms  such  as  thosc 
produced  by  certain  encephalic  lésions,  by  tumours  for  instance, 
and  so  closely  resembling  them  that  diagnosis  becomes  extremely 
difficult.  At  the  same  time,  I  shall  point  ont  to  you  what  advan- 
tages  you  may,  under  such  circumstances,  dérive  from  an  ophthal- 
moscopic  examination. 

In  the  second  place,  according  to  some  ophthalmologists,  the 
optic  lésion  proper  to  ataxia  may,  in  a  certain  uumber  of  cases, 
précède  ail  the  other  symptoms,  and  of  itself  constitute  the  whole 
disease,  often  during  many  long  years.  Now,  nothing  is  bctter 
established,  in  my  opinion,  as  I  hâve  already  indicated,  than  the 
accuracy  of  this  proposition.  If  that  be  correct,  it  would  con- 
sequently  be  of  the  highest  importance,  as  you  perceive,  to  know 
precisely  the  characters  which,  according  to  the  authors  quoted, 
enable  us  to  recognise  the  amaurosis  of  ataxic  patients,  and  to 
distinguish  it  from  ail  the  other  forms  of  visual  decay. 

With  respect,  firstly,  to  the  isolated  existence  of  taheilc  amaurosis 
during  a  séries  of  years,  that  is  a  fact  the  reality  of  wdiich  may  be 
readily  demonstrated,  in  this  vast  hospital,  by  means  of  observa- 
tions made  on  a  large  scale.     I  believe  myself  in  a  position  to 


CLINICAL    CASES.  37 

déclare  tliat  a  great  majorïtij  of  the  loomen  who  are  admitted  into 
thèse  wards^  as  afîlicted  with  amaurotic  blindness,  sooner  or  later 
présent,  after  admittance,  more  or  less  manifest  symptoms  of  ataxia. 
Alreadj,  in  iny  lectures  of  1868,  I  laid  stress  on  tliis  point,  and 
my  further  observations  allow  me  to  coniirm  what  I  tlien  asserted 
in  référence  to  this.  I  might  quote  nu  mérous  cases  in  support  of 
my  statement,  but  I  shall  content  myself  with  summarising  the 
examples  which  are,  in  fact,  very  characteristic. 

1°.  Mil — (Salle  St.  Alexandre,  No.  12),  aged  ^^  years  ;  she 
entered  this  asylum  on  account  of  blindness,  in  1855.  Disorders 
of  vision,  accompanied  by  head-pains,  made  their  appearance  in 
1850.  Confîned  at  first  to  the  left  eye,  they  soon  invaded  the 
right.  At  the  end  of  a  year  the  blindness  was  complète.  Now, 
it  was  only  in  1860,  that  is  to  say,  ten  years  after  the  outset  of  the 
phenomena,  that  shooting  pains  appeared  for  the  first  time.  They 
soon  became  complicated  with  girdliug  pains  ;  since  then  the 
disease  has  remained  almost  stationary.  However,  symptoms  of 
motor  incoordination  began  to  show  themselves  a  few  months  ago. 

a°.  Coud —  (Salle  St.  Charles),  also  aged  about  ^^  years.  At 
the  âge  of  26,  that  is,  twenty-nine  years  ago,  she  suffered  from 
violent  darting  pains  in  the  orbit,  and  shortly  after  was  smitten 
with  blindness  first  in  the  left  eye,  then  in  the  right.  Three  years 
later  she  was  seized  with  lightning  pains  in  the  head  and  muscles, 
with  which  gastric  crises  were  associated.  The  disease  since  then 
has  undergone  no  aggravation. 

Thèse  cases,  gentlemen,  could  be  multiplied^  if  I  did  not  fear  to 
weary  you.  To  sum  up,  I  am  much  disposed  to  believe,  from  what 
I  hâve  seen,  that  amaurotic  patients,  the  cause  of  whose  blindness 
is  progressive  atroj^hij  of  the  painlla,  are  unlikely  to  escape  this 
inévitable  law. 

Hence  it  is  of  importance  to  be  able  to  recognise  the  identity, 
from  the  beginuing,  of  this  affection  of  tlie  optic  nerve  which,  ten 
or  fifteen  years  after  its  invasion,  shall  be  foUowed  by  ataxia  ;  in 
other  words,  to  be  able,  when  a  case  of  amaurosis  from  atrophie 
lésion  of  the  optic  nerve  is  before  us,  to  déclare  whether  ataxia  will 
almost  inevitably  follow,  sooner  or  later,  or  whether,  ou  the 
contrary,  the  affection  of  the  optic  nerve  will  remain  isolated. 

Let  us,  then,  investigate  the  characteristics  of  grey  induration  of 
the  optic  nerves,  and  examine  if  they  be,  in  reality^,  almost  infallible, 
as  we  are  told. 


38  CHARAOTEES   OF    ANATOMICAL  LESION. 

HT. 

Aîid  first,  one  word  concerning  tlie  anatomical  lésion  to  which 
attaches  the  visual  disorder  we  are  about  to  study. 

To  the  nakecl  eye  the  altération  of  the  optic  nerve  shows  itself 
under  the  form  of  a  grey  induration,  whose  appearance,  in  every 
respect,  recalls  that  of  spinal  fasciculated  sclerosis.  As  a  gênerai 
rule,  it  seems  to  commence  by  the  peripheral  extremity  of  the 
nerve,  and  then  to  extend,  in  a  progressive  manner,  towards  the 
central  parts.  The  optic  bands  (tsenise  thalami  optici)  are  also 
affected,  in  their  turn,  at  a  given  moment,  and  sometimes  even  the 
corpora  geniculata.  Beyond  this  point  ail  trace  of  the  altération  is 
lost.  It  is  remarkable  that  this  should,  as  you  perceive,  follow  a  cen- 
tripelal  course  in  the  optic  nerve,  whilst  corresponding  lésions  of  the 
spinal  nerves  follow,  on  the  contrary,  a  centrifugal  course. 

Histological  o'esearch,  in  its  turn,  allows  us  to  recognise  new 
analogies  between  the  grey  induration  of  the  optic  nerves  and  tabetic 
spinal  sclerosis.  In  connection  with  this  subject  it  is  to  be  remem- 
bered  that  the  optic  nerves  are  much  nearer,  in  texture,  to  the  white 
substance  of  the  nerve  centres  than  ail  the  other  nerves.  Thus,  we 
find  in  the  optic  nerves,  as  M.  Leber  has  pointed  out,  stellate  con- 
nective  cells,  and  a  fibroid  reticulum.  I  should  add  that  thenerve- 
tubes  which  form  them  are  very  fine,  very  délicate,  and  conse- 
quently  hâve  much  analogy  with  the  nerve-tubes  of  the  ence- 
phalon. 

The  lésion  which  constitutes  the  grey  induration  is  also  marked 
in  the  optic  nerve,  as  in  the  spinal  cord,  by  the  fibrillary  metamor- 
phosis  of  the  neuroglia  and  the  concomitant  disappearance,  first,  of 
the  medullary  cylinder,  and  then  of  the  axis  cylinder.  To  say  which 
of  the  two  phenomena  précèdes  the  other  is  a  difficult  thing.  I 
incline  greatly  to  the  belief  that,  just  as  in  the  spinal  cord,  the 
nerve-tube  is  affected  in  the  first  instance,  before  the  connective 
matrix.  In  this  way  we  might  explain  why,  in  tabetic  lésion  of 
the  optic  nerves,  the  nerve-element  undergoes  a  comparatively 
much  more  complète  and  rapid  destruction  than  that  which  takes 
place  in  disseminated  sclerosis,  in  which  affection  the  axis  cylinders 
do,  in  reality,  persist  for  a  much  longer  time.  The  grey  induration 
of  the  optic  nerves,  in  locomotor  ataxia,  might,  accordingly,  be 
designated  by  the  name  of  parenchymatous  neuritis. 

However  this  may  be,  we  cannot  yet  hâve  recourse  to  histology 


OPHTHALMOSCOPIO   OHAEACTERS.  39 

for  the  distinctive  characters  ;  for  in  this  respect  tliere  is  a  very 
great  resemblance  between  the  induration  occurring  in  the  optic 
nerve,  in  conséquence  of  the  neuritis  connected  with  cérébral  tumours 
(opiic  neuritis),  and  the  grey  induration  of  the  same  nerve  in  the 
case  of  tabetic  patients.  Let  us,  then,  look  for  more  décisive  data 
in  cHnioal  observation. 

In  the  first  place,  let  us  describe  the  ophthalmoscopic  characters 
which,  in  fact,  up  to  a  certain  point,  answer  to  an  anatomical 
démonstration  in  the  living  subject.  I  shall  very  briefly  remind 
you  of  the  appearance  of  the  optic  papilla  in  the  normal  state. 

You  hâve  not  forgotten  the  somewhat  oval  form  which  it  présents, 
its  well-defined  and  distinct  borders,  the  eup-shaped  dépression  in 
its  centre,  finally,  the  slightly  rosy  tint  which,  on  the  other  hand, 
distinguishe^  its  peripheral  portion,  and  which  is  due  to  the 
présence  of  the  vasse  proprise  enclosed  in  the  substance  of  the 
optic  nerve.  With  respect  to  the  vessels  of  the  papilla,  you  know 
that  they  consist  of  two  veins  and  one  artery,  the  latter  much  ii^ 
ferior  in  volume  to  the  former,  and  besides,  easily  identified  by  the 
dichotomous  divisions  which  it  présents. 

Now,  hère  is  an  account  of  how  ail  thèse  peculiarities  are  found 
modified  in  a  case  of  progressive  grey  induration. 

The  papilla  shows  no  change,  neither  in  its  form  nor  in  its 
dimensions  ;  its  borders  are  still  well  marked.  The  vessels  remain 
what  they  were  before,  only,  contrary  to  what  happens  in  the 
normal  state,  we  can  no  longer  follow  them  as  they  penetrate  at  a 
certain  distance  into  the  substance  of  the  papilla,  on  which  they 
appear  to  be  simply  laid.  Nothing,  indeed,  is  noticed  radically 
différent  from  the  normal  condition  ;  but  hère  is  the  décisive 
characteristic.  In  conséquence  of  the  change  of  texture  which  the 
optic  nerve  has  undergone,  and,  above  ail,  because  of  the  disappear. 
ance  of  the  meduUary  cylinder,  the  papilla  has  ceased  to  be  trans- 
parent ',  it  strongly  reflects  the  light,  on  the  contrary,  and  no 
longer  allows  us  to  preceive  the  vasse  proprise  in  its  substance.  It 
follows  that  it  shows  no  more  its  normal  rosy  tint,  but,  on  the 
contrary,  a  white,  chalky  colour,  of  pearly  aspect. 

Such,  gentlemen,  is  the  characteristic  which  we  must  sedulously 
bear  in  mind,  for,  that  alone,  when  it  is  distinctly  manifest,  is  suffi- 
cient  to  specify  tabetic  amaurosis  and  to  clear  up  the  situation  in  a 
décisive  manner.  Still  it  is  proper  not  to  neglect  the  functional 
disorders  which,  also,  hâve  an  importance  of  their  own.     They  can. 


40  CHARACTERS    OF    TABETÏC    AMAUEOSIS. 

in  fact,  contribute  potently  to  form  tbe  diagnosis,  in  cases  where  the 
ophthalmoscopic  signs  are  little  marked,  by  adding  more  weight 
to  tbe  impression  made  upon  tbe  observer.  Besides,  amongst 
thèse  functional  dérangements,  there  are  some  wbicb,  even  in  tbe 
absence  of  tbe  opbtbalmoscopic  signs,  cause  the  nature  of  tbe 
disease  to  be,  to  a  certain  extent,  pre-judged. 

I  will,  in  tbe  first  place,  point  to  the  concentric  and  unilatéral 
limitation  of  the  visual  field,  a  functional  dérangement  which  is  not 
found  in  ojitic  neuritis,  and,  in  the  second  place,  to  the  more  or  less 
marked  contraction  of  the  pupils,  a  striking  contrast  with  what 
takes  place  in  optic  neuritis,  where  the  pupils,  on  the  contrary, 
appear  dilated. 

We  should  mention  a  symptom  which,  according  to  certain 
authors  (Galezowski,  Benedikt),  is  in  some  degree  specially  charac- 
teristic,  namely,  a  peculiar  form  of  achromatopsia  thus  distinguished  : 
1°,  loss  of  perception  for  secondary  colours  (i  and  5  of  M. 
<Galezowski's  scale)  ;  a°,  loss  of  perception  for  red  and  green,  the 
perception  for  yellow  and  hlue  persisting,  on  the  contrary,  to  a  higb 
degree  and  for  a  long  time.  Thèse  signs  may  already  bave  shown 
tbemselves  very  strongly  marked,  at  a  period  when  loss  of  visual 
acuity  is  incomplète,  and  the  patient  can  still  read  large  type. 

I  should  add  that  the  commencement  of  thèse  phenomena  in  one 
eye,  and  the  prolonged  localisation  of  the  lésion  in  this  same  eye, 
are  quite  the  inverse  of  what  is  observed  in  optic  neuritis.  Again, 
in  tabès,  tbe  évolution  of  visual  disorders  is,  in  the  immense 
majority  of  cases,  slow,  graduai,  progressive  ;  whilst  in  optic 
neuritis  their  invasion  often  enougb  bappens  in  an  almost  sudden 
manner. 

The  other  functional  disorders  which  it  remains  for  us  to  notice 
are  rather  of  a  kind  to  obscure  the  diagnosis  ;  but,  on  that  very 
account,  they  also  deserve  to  be  remarked.  Such  are  tbe  con- 
tinuons or  almost  continuons  head-pains  which  are  cbiefly  situate 
in  the  forehead  and  at  the  nape.  With  thèse  permanent  pains  are, 
in  many  cases,  associated  lightning  pains,  occurring  in  paroxysms, 
and  occupying  the  course  of  the  branches  of  the  fifth  pair.  In 
tbe  paroxysm,  the  patients  complain  of  feeling  as  if  the  globe  of 
tbe  eye  were  being  torn  out. 

Apart  from  the  head  pains,  which  are  a  somewhat  common-place 
symptom,  the  phenomena  which  bave   just  been   described  form 
when   taken  together,  an   almost  characteristic   syndroma.     They 


NEUKO-RETINITIS.  41 

allow  tabetic  amaurosis  to  be  easily   distinguished^  for  instance, 
from  the  amaurosis  accompanying  disseminated  sclerosis. 

Embolism  of  the  central  artery  of  the  retina  gives  rise,  in  the 
long  ruu,  to  ophthalmoscopic  appearances  which  simulate  those  of 
the  tabetic  papilla.  There  are,  however,  decidedly  distinctive 
characters,  which  you  will  find  set  out  in  spécial  works.  The 
suddeu  invasion,  in  the  case  of  embolism  and  the  customary  coexist- 
ence of  hemiplegia  and  heart  disease,  will  not,  besides,  leave  the 
observer  long  in  doubt. 

I  shall  only  make  a  passing  mention  of  the  lésion  of  the  optic 
nerve  which  sometimes  supervenes  in  glycosuria  and  syphilitic 
retino-choroiditis,  as  being  equally  capable  of  producing,  to  a 
certain  point,  the  appearance  of  tabetic  papillary  atrophy.  Pinally, 
in  gênerai  paralysis,  we  occasionally  observe  a  lésion  of  the  papilla 
which  differs  in  nothing  esseutial  from  that  seen  in  ataxia  ;  but  we 
hâve  already  taken  care  to  point  out  that  tabetic  spinal  lésions  are 
fouud,  in  some  cases,  connected  witli  gênerai  paralysis,  and  this 
circumstance,  perhaps,  may  explain  the  fréquent  occurrence  of  pro- 
gressive papillary  atrophy  in  diffuse  chronic  meningitis. 

I  confine  myself  to  a  succinct  mention  of  thèse  several  affections, 
as  I  propose  to  request  your  entire  attention  to  the  objective 
symptoms  which  the  altération  of  the  optic  nerve  produces,  and 
which  are  known  as  optic  neitritis  or  neuro-retinitis,  for  there,  in 
point  of  fact,  is  the  knot  of  the  situation. 

Undoubtedly,  there  are  analogies,  on  the  one  hand,  between  the 
concomitant  symptoms  of  neuro-retinitis  and  those  which  accompany 
tabetic  atrophy  ;  and,  on  the  other  hand,  between  the  appearance  of 
the  papilla,  which  dénotes  papillary  atrophy  consécutive  on  optic 
neuritis,  and  that  of  tabetic  amaurosis.  But  there  are  likewise 
distinguishing  characters  for  each  of  them,  and  the  knowledge  of 
thèse  characters  will  enable  us  to  render  the  diagnosis  certain. 

IV. 

In  order  to  attain  this  object,  we  must  now  enter  into  some 
détails,  with  respect  to  neuro-retinitis  and  the  circumstances  amid 
which  it  arises.     Of  thèse,  two  deserve  particular  notice  : 

1°.  (a)  Blindness,  so  common  in  cases  of  cérébral  tumours,  since 
it  occurs  in  nearly  half  the  cases  (Friedreich  and  Ladame),  appears 
for  the  most  part  to  be  caused  by  neuro-retinitis. 


43  rOEMS    OF  NEURO-RETINITIS. 

(b)  Meningitis  of  the  base,  sypliilitic  or  not,  is  likewise  tolerably 
often  tlie  occasion  of  neuro-retinitis  ;  and,  in  sucli  cases,  the  pro- 
gnosis  is  very  différent  froin  what  it  is  on  the  hypothesis  of  cérébral 
tumours.  If  we  hâve  to  do  with  a  syphilitic  lésion,  in  particular, 
the  blindness  need  not  be  inevitably  progressive,  and  sight  may 
persist,  at  least  to  a  certain  degree. 

Authors  admit  two  principal  forms  of  neuro-retinitis.  In  the 
first  place,  cornes  neuro-retinitis  par  étranglement  {Stauungs  Papille 
of  the  Germans,  and  ^'choked  dise"  of  British  authors).  It  is 
anatomically  characterised  by  a  frequently  enormous  tuméfaction  of 
the  papilla,  caused  by  simple  congestion  with  serous  exudation. 
This  form  seems,  especially,  connected  with  the  existence  of  intra- 
cranial  tumours.  According  to  Von  Graefe,  the  symptoms  which 
characterise  it  resuit  from  the  augmentation  of  intra-cranial  pres- 
sure. But  it  appears  to  be  proved  that,  besides  the  papilla,  the 
nervc  itself  may  be  affected  throughout  its  whole  extent,  and  pré- 
sent a  certain  degree  of  tuméfaction  and  softening,  or  even  exhibit 
the  anatomic  characters  of  inflammatory  optic  neuritis.  This,  at 
ail  events,  is  what  seems  to  resuit  from  the  observations  of  MM. 
Hulke,  Albutt,  and  some  others.  There  would  exist,  according  to 
this,  a  sort  of  transition  between  the  two  forms  of  neuro-retinitis. 

2°.  The  second  of  thèse  forms  is  usually  known  by  the  name  of 
descending  neuro-reiinilis.  Some  ophthalmologists,  and  Von  Graefe 
amongst  them,  assert  that  this  form  is  connected,  in  a  spécial 
manner,  with  meningitis,  and  that  if  it  be  sometimes  associated 
with  tumours,  from  accompanying  them,  this  happens  because  basai 
meningitis  exists  at  the  same  time.  If  it  is  possible  to  quote 
three  cases  of  Von  Graefe,  as  mentioned  by  Mr.  Albutt,  in  support 
of  this  opinion,  we  should  state  that  a  récent  fact,  observed  in  this 
hospital,  has  occurred  to  contradict  it,  or  at  least  to  abrogate  its 
absolute  rule. 

The  case  is  that  of  the  patient  Ler — ,  in  whom  the  symptoms  of 
optic  neuritis  with  atrophy  of  the  nerve  were  observed.  Thèse 
lésions  were  connected  with  the  existence  of  a  sarcomatous  tumour 
oecujjying  the  left  occipital  lobe  of  the  brain.  The  cerebellar  roof 
had  been  pushed  back  ;  the  mesocephalon,  as  well  as  the  optic 
bands  and  the  tubercula  quadrigemina,  were  greatly  flattened. 
Well,  in  this  case,  in  which  intra-cranial  pressure  was  evidently 
exaggerated  (the  hypertrophy  and  flattening  of  the  convolutions 
leaving   no  doubt   in   this  respect),  and  in  which   probably  the 


CLINICAL    CHARACTEES    OF    OPTIC    NEURITIS.  43 

clioking  of  tlie  papilla  had  taken  place,  at  a  certain  period,  the 
optic  nerves  were  grey,  atrophied,  in  one  word,  sclerosed  through- 
out  their  wliole  exient.     No  traces  of  meningitis  existed. 

This  scierons  atrophy  characterises  the  second  form,  or,  if  it  be 
preferred,  the  second  degree  of  optic  neuritis.  Anatomically,  we 
hâve  an  interstitial  neuritis,  witli  fibrillary  substitution,  consécutive 
destruction  of  the  nerve  éléments.  The  morbid  process  hère 
assumes  a  more  acute  course  than  in  the  case  of  tabetic  optic 
neuritis  ;  multiplication  of  nuclei  is  more  marked,  exudation  more 
abuudant,  and  there,  in  short,  lies  the  whole  différence. 

V. 

Let  us  now  inquire  what  are  the  characters  presented  to  the 
clinical  observer  by  the  papilla,  in  thèse  two  forms,  or,  if  yoa 
please,  in  thèse  two  periods  of  optic  neuritis,  and  contrast  them  with 
the  characters  which  distinguish  the  tabetic  papilla. 

A.  With  respect  to  the  choked  papilla  nothing  can  be  more 
simple.  The  papilla,  in  fact,  then  exhibits  a  tuméfaction,  a  swell- 
ing,  manifest  at  the  first  glance. 

The  borders,  ill-defined  besides,  are,  as  it  were,  effaced  by  an 
exudation  apparently  spread  both  over  the  papilla  and  around  it. 
This  exudation  is  of  a  reddish-grey  colour.  Hère  and  there  the 
central  vessels  are,  to  ail  appearauce,  interrupted.  This  pheno- 
menon,  very  marked  as  regards  the  veins,  is  less  évident  with  respect 
to  the  arteries,  which  are  small  in  comparison.  The  capillaries  are 
well  developed,  at  least  at  a  certain  period.  This  assemblage  of 
phenomena  is  already  very  striking  ;  but  the  functional  symptoms 
also  merit  observation.  I  shall  confine  myself  to  noticing  the 
following  features  : — Both  eyes  are  usually  smitten  at  the  same  time, 
the  invasion  is  sometimes  sudden,  there  is  no  concentric  diminution 
of  the  field  of  vision;  finally,  no  chromatic  modification  is  re- 
marked. 

B.  What  now  are  the  characters  of  optic  neuritis,  considered  in 
its  second  form  ?  The  papilla,  enlarged  after  a  manner,  shows  itself 
with  fringed  borders,  irregular  and  ill-defined.  You  would  say  it 
was  surrounded  by  a  sort  of  cloud.  Because  of  the  opacity  which 
affects  the  optic  nerve  the  capillaries  and  the  rosy  tint  seem 
effaced.  The  blood-vessels  are  tortuous  and  winding,  especially  the 
veins,  which  appear  interrupted,  as  if,  hère  and  there,  they  had  been 
eut. 


44  CLINICAL    FAOTS. 

Thèse  are  characters,  gentlemen,  whicli  are  never  quite  effaced, 
and  which  contrast  with  the  ophthalmoscopic  characters  assigned 
lo  the  tabetic  papilla.  As  regards  functional  symptoms,  they  are 
similar  to  those  of  choked  papilla. 

VI. 

It  is  not  enough  to  hâve  described  the  characters  which,  whether 
functionally  or  by  ophthalmoscopic  examination,  distinguish  tabetic 
altération  of  the  papilla  from  that  attaching  to  optic  neuritis.  It  is 
indispensable,  also,  to  show,  in  the  living  subject,  what  use  we  can 
make  of  thèse  facts  for  the  better  advantage  of  the  diagnosis.  I 
shall  confine  myself  to  one  example. 

Quite  recently  we  had  two  patients,  side  by  side,  in  our  wards. 
One  of  them,  named  Deg — ,  I  showed  to  you  as  a  spécimen  of 
abortive  ataxia,  with  fulgurant  and  gastric  crises,  unaccompanied 
by  motor  incoordination.  The  other,  named  Ler — ,  succumbed 
a  few  days  ago.  The  first-mentioned  is  an  ataxic  patient,  and  no 
one  can  doubt  the  diagnosis,  tliough  the  anatomic  criterion  be 
lacking  ;  the  second  had  a  tumour  in  one  of  the  occipital  lobes  of 
the  brain. 

But,  you  will  ask  me,  what  connection  is  there  between  a  tumour, 
occupying  the  occipital  lobe,  and  a  case  of  ataxia,  in  its  first  stage  ? 
Thèse  are,  in  truth,  two  diseases  which  are  not  usually  mentioned 
together,  because  they  differ  by  very  decided  characters.  Well, 
gentlemen,  it  behoves  us  not  to  dépend  too  much  upon  thèse 
characters;  they  may  deceive  you.  And,  in  point  of  fact,  the 
combination  of  symptoms  in  our  two  patients  was  such  that,  for  a 
long  time,  there  was  great  perplexity,  and  the  diagnosis  remained 
absolutely  uncertain.  Eor  me,  it  is  not  a  thing  of  doubt  that  cer- 
tain cases  of  cérébral  tumours,  which  are  of  course  great  exceptions, 
should  be  connected,  clinically,  with  locomotor  ataxia,  A  statement 
of  the  two  cases,  just  mentioned,  will,  however,  afford  a  better 
démonstration  than  could  be  got  from  a  lengthy  commentary. 

The  patient  Deg —  présents  the  following  symptoms  :  intense 
rémittent  cephalalgia  in  occiput  and  forehead  ;  pains  in  the  eyeballs, 
complète  blindness  on  both  sides  ;  nearly  constant  pains  in  the  nape, 
which  appear  to  spread  down  the  entire  length  of  one  arm  ;  fits  of 
vomiting  constituting  true  gastric  crises  and  accompanied  by  exas- 
pération of  cephalic  pains  ;  finally,  painful  fulgurations  in  ail  the 
limbs  coming  on  in  paroxysms. 


CLINICAL    FACTS.  45 

The  symptoms  observed  in  the  case  of  Ler —  require  a  more 
minute  description.  We  find  :  complète  blindness,  supervening 
gradually  (sudden  invasion,  you  perceive,  is  nowise  necessary  in 
optic  neuritis)  ;  intense  cephalalgia,  occupying  the  forehead  and 
occiput,  well  nigh  continuous,  but  liable  to  be  exasperated  by 
paroxysms  ;  acute  pains  in  the  eyes,  subject  to  remissions  and 
exacerbations  ;  vomiting  coming  on  in  paroxysms,  just  as  in  the 
case  of  Deg — ,  and  occasionally  persisting  for  some  days  ;  ûnally, 
pains  in  the  extreraities. 

Thèse  pains,  which  constitute  an  exception  to  the  rule  I  men- 
tioned  at  the  beginning  of  this  lecture,  when  describing  tabetic 
pains,  displayed  in  an  unmistakable  manner  the  characteristic 
peculiarities  of  shooting  pains.  Over  a  score  of  times,  in  the  notes 
taken  down  from  the  sincère  description  of  the  patient,  and  regis- 
tered  at  the  very  moment  of  the  paroxysm,  we  find  it  stated  that 
thèse  pains  come  on  suddenly,  like  flashes  of  lightning,  that  they 
affect  but  a  point,  either  near  the  joints  (knee  or  wrist),or  on  the  body 
of  the  limbs,  and  that  they  are  accompanied  by  a  sort  of  starting 
of  the  members  attacked.  When  exaspération  of  thèse  pains  and 
of  the  cephalalgia  occurs  then  the  paroxysms  of  vomiting  come  on. 
In  addition  to  ail  thèse  symptoms  we  should  add  a  vertébral  pain, 
spreading  round  the  body  and  simulating  the  girdle  pain.  Thèse 
peculiar  pains,  so  remarkably  manifest  in  our  patient,  do  form  an 
absolutely  exceptional  fact  in  cases  of  cérébral  tumours.  Thus,  in 
2^^  cases  M.  Ladame  mentions  twenty-three  times  the  existence  of 
rheumatoid  pains  in  différent  parts  of  theextremities.  Undoubtedly, 
it  is  but  very  rarely  that  thèse  assume  the  character  of  fulgurant 
pains  ;  nevertheless,  this  author,  though  not  indeed  emphasising  it, 
points  out  the  présence  of  more  or  less  acute  pains,  recurring  in 
paroxysms,  and  flitting  frequently  from  one  point  to  another. 

However  that  may  be,  this  singular  complication  is  established 
in  a  peremptory  manner,  were  it  but  by  the  case  of  Ler —  alone. 
And  to  account  for  it,  you  cannot  appeal  to  some  tabetic  complica- 
tion, for  the  posterior  columns,  which  were  carefully  examined  at 
the  autopsy,  hâve  been  found  perfectly  healthy. 

Well,  then,  gentlemen,  with  such  a  case,  and  in  ail  probability 
cases  of  this  kind  will  occur  again,  is  not  the  question  of  diagnosis 
a  most  embarrassing  one  ?  Allow  me  also  to  inform  you,  in  order 
to  add  to  the  interest  of  the  situation,  that  in  the  tumour  case  there 
was  titubution,  whilst  in  the  ataxic  case  there  was  no  trace  of  it. 


46  CLINICAL    TACTS. 

Now,  in  this  difficulty,  ilie  oplitlialmoscopic  art  came  to  give  us 
its  décisive  aid.  I  place  before  your  observation  two  drawings^  done 
after  nature^  which  I  owe  to  tlie  kindness  of  M.  Galezowski  :  one 
of  them  represents  the  papilla  of  Deg — _,  and  you  can  recognise  in 
it  ail  the  characters  of  the  tahetic  papïlla  ;  the  other  shows  the 
papilla  of  Ler — ,  where  atrophy,  consécutive  on  optic  neuritis,  i& 
seen  with  ail  its  distinguishing  signs. 

After  that  examination  ail  difficulty  ceased  immediately.  It 
became  évident  that  Ler —  was  affected  by  cérébral  tumour,  as  the 
autopsy  bas  verified  ;  with  respect  to  Deg —  she  is  ataxic.  The 
necropsy  some  day  will  décide,  and  justifj  us,  I  bave  no  doubt. 

This  example,  chosen  from  amongst  so  many  others,  will  suf&ce, 
I  hope,  to  bring  out,  before  your  eyes,  the  interest  pertaining  to 
ophthalmoscopic  study  in  the  clinical  observation  of  diseases  of  the 
nerve  centres.  Consequently,  gentlemen,  I  could  not  too  strongly 
recommend  you  to  seek  in  the  application  of  Helmholtz's  mirror 
the  invaluable  assistance  which  it  is  capable  of  yielding  in  such 
circumstances.  Cases  abound  in  this  hospital,  and,  in  a  very  short 
time,  you  will  be  able,  with  alittle  practice,  to  put  yourselves  abreast 
of  the  fundamental  facts. 

M.  Galezowski  is  good  enough  to  give  us  his  assistance,  and  ofFers, 
by  means  of  the  spécial  instrument  which  he  bas  invented,  and  which 
renders  démonstration  so  much  more  easy,  to  render  évident  to  ail 
of  you  those  particular  facts  upon  which  I  hâve  dwelt  thus  minutely 
to-day. 


LECTURE  IV. 

ON  SOME  VISCERAL  DERANGEMENTS  IN  LOCOMOTOR  ATAXIA. 
ARTHROPATHIES  OF  ATAXIC  PATIENTS. 

SuMMARY. — Bisorders  of  the  genïto-urinary  organs.  Freqtient 
désire  to  micUirate  ;  satyriasis  ;  rectal  tenesmus .  Oculo-pupil- 
lary  symptoms.  Permanent  accélération  and  dicroiism  of  the 
puise. 

ArtJiropathy  of  ataxic  patients  :  ils  frequency.  CaseSi 
This  artliropatJiy  is  developed  at  a  but  slightly  advanced  period 
of  the  spinal  disease.  Prodromes.  Phases  of  ataxic  arthro- 
piathy.  Joints  attacked.  Its  spécial  characters.  Biagnosis 
hetween  the  arthropathy  of  ataxic  patients  and  dry  arthritis. 

Anatomical  lésions.  Arthropathies  consécutive  on  affections 
of  spinal  origin.  Mechanism  of  production  of  ataxic  arthro- 
pathies. Lésions  of  the  anterior  cormia  of  the  grey  substance. 
Desideratum. 

Gentlemen, — It  is  my  intention  to  terminate,  this  morning, 
the  history  of  those  anomalies  which  are  most  commonly  met  with. 
in  the  first  period  of  progressive  locomotor  ataxia. 

I. 

In  one  of  our  last  meetings,  I  spoke  to  you  concerning  the 
gastric  crises,  and  I  showed  you  the  important  diagnostic  part 
which  this  phenomenon  plays,  when  combined  with  certain  cephalic 
symptoms,  such  as  cephalalgia  and  amaurosis  from  grey  induration 
of  the  optic  nerve.  It  is  proper,  in  connection  with  thèse  gastric 
crises,  to  mention  some  other  viscéral  affections  which  may  alsa 
coexist  alone  with  the  lightning  pains,  in  the  period  named  after 
them. 

A.  Thus,  it  is  not  an  exception  to  find,  during  a  fulgurant  attack. 


48  DISORDERS    OP    GENITO-URINARY    ORGANS. 

that  certain  disorders  of  i\\e  genito-urinary  organs  superveup,  which 
hâve  an  importance  of  their  own.     Such  are  ; 

1°.  Fréquent  désire  to  micturate,  witli  painful  émission  of 
urine. 

2°.  SatT/riasis,  onwliich  Trousseau  laid  stress  ; — sexual  disorder, 
of  this  class,  lias  scarcely  been  observed  except  in  the  maie. 
The  symptoms  which  characterise  it  in  the  maie  consist  in  fréquent 
and  incomplète  érections,  vvith  prématuré  émission,  &c.  Analogous 
phenomena  may  also  be  manifested  in  the  female,  as  M.  Bouchard  and 
myself  hâve  pointed  ont.  We  hâve  found  them  especially  marked  in 
the  woman,  named  Bar — ,  whom  we  observed  during  a  long  time 
in  La  Salpêtrière,  and  who,  on  the  occurrence  of  fulgurant  crises, 
often  experienced  voluptuous  sensations,  similar  to  those  of  coition, 
and  accompanied  by  au  abundant  vulvo-vaginal  sécrétion. 

3°.  The  rectum  may  also  be  affected  by  singular  sensations.  We 
shall  hère  again  refer  to  the  case  of  the  patient,  M.  C — ,  of  whom 
we  hâve  already  spoken  ;  even  before  the  shooting  pains  had  made 
their  appearance,  he,  from  time  to  time,  experienced  sudden  sensa- 
tions in  the  anus  and  rectum,  which  he  compared  to  what  would  be 
caused  by  the  forcible  thrusting  of  a  long  voluminous  object  into 
the  rectum.  Thèse  sensations  supervened  suddenly  and  disappeared 
rapidly.  At  times,  a  need  of  expulsion  was  likewise  felt,  which 
was  followed  by  repeated  involuntary  évacuations  of  fpeces,  This 
kind  of  painful  spasm  of  the  intestine  existed  in  this  patient  for 
nearly  eight  months  before  the  pains  in  the  extremities  began  to 
appear. 

You  will  readily  understand  what  interest  thèse  epiphenomena 
may  acquire,  in  certain  circumstances  where  diagnosis  has  remained 
uncertain. 

B.  There  is  some  reason  to  suppose  that  the  great  sympathetic 
plays  a  part  in  the  production  of  thèse  viscéral  crises,  as,  for 
brevity's  sake,  I  shall  call  them  ;  and  this  is,  perhaps,  the  place  to 
point  out  to  you  certain  other  phenomena  where  the  great  sym- 
pathetic is  clearly  at  work.  I  mean  the  oculo'pnpillary  symptoms 
mentioned,  for  the  first  time,  I  believe,  by  Dr.  Duchenne  (de 
Boulogne).  Participation  of  the  cervical  sympathetic  is  in  question 
hère. 

Prom  the  first  period  of  the  ataxia  it  is  common  to  fmd  ajmpil- 
lary  ineqnaliti/,  and,  on  the  same  side  with  the  more  contracted 
pupil  (myosis),  there   occasionally  are  phenomena  which    reveal 


ACCELERATION  OF  THE  PULSE.  49 

the  paralytic  state  of  the  vaso-motors  :  the  cheek  is  red  ;  the  eye, 
which  is  injected,  présents  a  sort  of  chemosis  ;  finally,  there  is  a 
relative  increase  of  température.  During  the  fulgurant  attack,  the 
coutracted  pupil  dilates^  and  the  signs  of  vaso-motor  paralysis 
momentarily  disappear. 

c.  Beside  thèse  phenomena  I  shall  place  the  permanent  accéléra- 
tion of  the  puise  which,  as  I  hâve  shown,  is  frequently  found  in 
ataxic  patients  (90 — 100),  and  the  habituai  dicrotism  to  which 
M,  Eulenberg  has  called  attention. 

Pinally,  we  must  mention  the  genuine  fever  which,  at  the  onset 
of  the  ataxia,  sometimes  accompanies  the  fulgurant  crises,  as  I 
hâve  many  times  remarked  (in  13 — ^s  case  in  particular),  and  to 
which  testimony  is  likewise  borne  by  Dr.  Finckelburg,  director  of 
the  hydropathic  establishment  of  Godesberg,  and  Professer  Rosenthal, 
of  Vienna.  But,  I  cannot  deal  lengthily  with  thèse  différent 
phenomena  which,  however,  hâve  an  interest  of  their  own.  I 
désire,  at  présent,  to  insist  upon  an  affection,  whose  existence  I 
hâve  pointed  out,  and  which  I  am  accustomed  to  designate,  in 
order  to  prejudge  nothing,  by  the  name  of  arthropaihj  of  ataxic 
patients. 

II. 

To  my  mind,  and  I  hope  to  make  you  share  my  way  of  looking 
at  it,  we  hâve  hère  one  of  the  manifold  forms  of  spinal  arthropathy. 
What  is  spinal  arthropathy,  some  amongst  you  may  ask  ?  I  hâve 
proposed  to  designate  by  this  name  a  whole  group  of  articular 
affections,  which  appear  to  dépend  directly  on  certain  lésions  of  the 
spinal  cord,  with  which,  consequently,  they  should  be  connected 
as  symptomatic  affections.  The  irritative  lésions  of  the  spinal 
cord,  especially  those  which  occupy  the  grey  substance,  react 
sometimes,  you  are  aware,  ou  the  periphery,  and  détermine  varions 
nutritive  disorders,  either  in  the  skin  or  in  the  deeper  parts,  such  as 
the  muscles.  The  bones  and  articulations  do  not  appear  to  escape 
this  law.  It  follows  that  the  arthropathies  of  locomotor  ataxia 
would  be,  accordiiig  to  my  judgment,  one  of  the  forms  of  thèse 
articular  affections,  developed  under  the  more  or  less  direct  influence 
of  lésion  of  the  spinal  centre. 

Hère,  it  may  not  be  useless  to  make  you  remark  that  ail  the 
articular  affections  which  supervene,  in  a  patient  attacked  with 
locomotor  ataxia,  do  not  necessarily  come   within   the  following 

VOL.    II.  4 


'50  ARTHEOPATHY  OF  ATAXIA. 

description.  Thus,  it  is  not  rare  to  see  nodose  rlieumatism,  common 
dry  arthritis,  coincide  witli  ataxia.  Then^  and  on  this  point  I 
would  insistj  tliese  rheumatic  affections  sliow  themselves  with  their 
accustomed  sjmptoms.  Ataxic  arthropatliy,  on  the  contrary,  is 
evolved  with  clinical  characteristics^  altogether  its  own,  as  you 
will  soon  see,  which  cause  it  to  constitute  a  reallj  distinct  disorder. 

I  would  also  add  that  there  is  no  question  hère  of  an  extremely 
rare  and  exceptional  phenomenon.  I  can  show  you  five  examples  of 
thèse  arthropathies  in  about  fifty  ataxic  patients,  whoui  I  know  in 
this  refuge.  I^ive  cases  in  fifty,  is  aiready  a  respectable  number. 
Taking  my  own  expérience,  I  hâve  observed  this  complication  of 
ataxia,  perhaps  thirty  times,  in  private  practice  and  in  hospital. 
Abroad,  also,  Drs.  Albutt  in  England,  Mitchell  in  America,  and 
Eosenthal  in  Vienna,  hâve  recorded  analogous  instances.^ 

Let  us  first,  gentlemen,  consider  the  clinical  side  of  the  question. 
My  friend,  M.  Bail,  to  whom  several  important  works  on  this 
question,  are  due,  proposes  to  distinguish  in  ataxic  arthropatliy  : 
1°,  early  development,  and,  3°,  late  development.  In  my  opinion, 
the  arthropathy  in  question  is  always  an  early  phenomenon,  that 
is,  a  phenomenon  of  the  initial  period  of  the  spinal  disease.  To  be 
more  précise,  I  will  say  that,  in  the  natural  évolution  of  the 
disease,  it  occurs,  generally  at  ail  events,  at  an  intermediate 
epoch,  between  the  period  termed  prodromic  and  the  period 
of  incoordination.  If  the  affection  sometimes  appears  at  a  late 
epoch,  as  may  certainly  occur,  it  always  shows  itself  in  the 
upper  extremity,  in  the  shoulder,  elbow,  or  wrist.  Now,  you 
know  that  posterior  spinal  sclerosis  may  be  quite  récent  in  the 
superior  régions  of  the  cord,  when  it  is  aiready  very  old  in  the 
dorso-lumbar  région.  Eemark,  gentlemen,  the  date  of  this  mani- 
festation, constant  and  regular  so  to  speak,  in  the  course  of  the  spinal 
disease,  for  there  we  find  a  preliminary  argument  of  some  weight 
in  favour  of  the  spécial,  not  to  say,  the  spécifie,  nature  of  the 
articular  lésion  I  am  speaking  of.  In  order  that  you  may 
thoroughly  realise  how  this  affection  présents  itself  for  observation, 
permit  me  briefly  to  describe  a  few  cases. 

*  The  question  of  tiie  arthropathies  of  ataxia  was  recently  brought  before 
the  Médical  Society  of  Berliû  (3oth  October),  in  connexion  with  a  case  pre- 
sented  by  Dr.  Ponfick,  noted  in  the  wards  of  Dr.  Westphal.  See  *  Berlin 
Klin.  Wochcnschrift,'  No.  46,  25,  November,  1872;  No.  47,  2nd  Deoember: 
see  also,  same  journal,  No.  53,  note  by  Professor  Hitzig  :  "  Eiuige  Bemer- 
kungeu  liber  die  Pragc  uach  dem  Ursprung  der  Arthritis  deformans." 


CLINIOAL  PACTS.  51 

First  case. — M.  B—  had^  in  1860,  attacks  of  liglitning  pains. 
In  1866,  one  morning,  on  awaking,  he  was  greatly  astonislied  to 
see  tliat,  without  the  slightest  forewarning,  liis  left  knee,  the  upper 
part  of  his  leg,  and  the  lower  part  of  the  thigh,  on  the  same  side, 
had  been  invaded  by  a  paiuless,  but  very  considérable  swelling. 
M.  Nélaton,  being  consulted,  recognised  the  présence  of  liquid  in 
the  synovial  cavity.  At  the  end  of  a  few  days,  cracking  sounds 
were  noticed  in  the  joint.  Five  or  six  months  after,  ail  had 
returned  to  the  normal  state.  Now,  remark  this  peculiarity, — at 
the  time  of  this  occurrence,  there  existed  no  sign  of  motor  inco- 
ordination. The  legs  were  not  flung  out,  and  no  mechanical  cause 
had  intervened.  ]\Jotor  incoordination  did  not  show  itself  until 
1866.  Later  on,  in  1870,  tlie  ataxia  having  advanced,  thearticular 
affection  had,  on  the  contrary,  disappeared  without  leaving  any 
trace. 

There,  gentlemen,  you  hâve  a  fine  spécimen  of  the  early  and 
benign  form  of  the  disease. 

Second  case. — A  provincial  apothecary,  who  came  to  consult  me 
last  year,  experienced  its  invasion  at  a  still  earlier  period  ;  for  the 
arthropathy,  which  hère  also  affected  the  knee,  had  come  on  with  the 
first  crises  of  shooting  pains.  And,  precisely  as  in  the  last  case,  the 
affection  is  not  now  discernible  by  any  sign,  and  the  incoordination, 
though  very  évident,  is,  nevertheless,  not  very  marked,  since  it  allows 
the  patient  to  dévote  himself,  with  ardour,  to  botanical  excursions. 

Third  case. — The  history  of  Dr.  X —  which  I  related  to  you,  in 
speaking  of  gastric  crises,  resembles  also  that  of  M.  B — .  At  a 
period,  when  the  disease  was  symptomatically  constituted  by  attacks 
of  shooting  pains  and  gastric  crises  only,  Dr.  X —  became 
aware  of  the  existence  of  a  hydarthrosis  of  the  knee,  with  gênerai 
swelling  of  the  extremity,  supervening  without  cause  appréciable — 
such  are  his  own  words.  There  was  no  local  pain,  and  it  was 
possible  for  him  to  walk,  although  with  a  little  difficulty.  The 
incoordination  did  not  begin  to  show  itself  until  five  or  six  months 
after  ;  it  was  then  I  saw  the  patient.  The  knee  still  contained  a 
small  quantity  of  fluid,  and  the  member,  which  remained  voluminous, 
presented  a  kind  of  engorgement,  of  induration  rather  than  œdema. 

Pourth  case. — I  shall  hère  recall  the  patient  Aub — ,  whom  I 
showed  to  you,  and  who,  also,  had  gastric  crises  and  lightning  pains, 
and  walked,  without  incoordination,  when  a  swelling  supervened  in 
the  right  hip,  which  caused  her  to  be  admitted  into  the  surgical 


53  CLINIOAL  FAOTS. 

wards.  The  left  bip  was  invaded  at  a  later  period,  when  this 
woman  was  in  our  halls  and  we  were  able  to  assist  at  the  develop- 
ment  of  this  second  arthropathy.  It  was  posterior  to  the  appear- 
ance  of  the  arthropathies  that  the  phenomena  of  incoordination 
manifested  themselves  in  each  of  the  extremities. 

The  patient  furnishes  us  with  a  relatively  rare  example  of  a 
tabetic  arthropathy  affecting  the  bip-joints.  I  believed  for  a  long 
time,  and  very  wrongly  as  you  see,  that  this  articulation  was 
always  spared  in  such  cases.  She  furnishes  us,  on  the  other  band, 
with  an  example  of  the  malignant  form,  that  is,  a  form  characterised 
by  rapid  disorganisation  and  luxation  of  the  joint.  It  is  proper  to 
contrast  this  example  with  the  first  three  cases  which,  I  repeat, 
belong  to  the  benignant  form  that  may  terminate  in  cure. 

Fiftb  case. — A  patient,  named  Mén — ,  in  whom  I  had  for  the 
first  time  noticed  gastric  crises  of  a  well-marked  character,  présents 
a  typical  arthropathy  of  the  shoulder  (of  ail  the  joints  of  the 
upper  extremity,  the  shoulder  is  the  most  frequently  affected). 
Mén —  for  many  years  had  been  confined  to  her  bed,  and  the 
incoordination  was  little  noticeable  in  the  superior  members,  when 
one  morning,  after  a  paroxysm  of  lightning  pains,  she  called  our 
attention  to  the  pathognomonic  tuméfaction  of  the  shoulder  and 
the  whole  extremity.  We  found  an  enormous  hydarthrosis,  with 
swelling  of  the  entire  limb,  and  in  spite  of  that,  the  patient  did  not 
complain  of  any  pain  ;  the  rectal  température  had  not  risen  ;  the 
puise  as  usual,  was  ]  00°^.  At  the  end  of  a  few  days,  we  could 
easily  perceive  the  existence  of  strong  cracking  sounds  in  the  joint. 
M —  then  informed  us  that  she  had  noticed  them  during  the 
seven  or  eight  days  preceding  the  outset  of  the  tuméfaction.  The 
swelling  passed  away  pretty  rapidly,  and,  soon  after,  a  luxation  of 
the  shoulder  backwards  took  place. 

This  case  belongs,  of  right,  like  the  previous  one,  to  the  de- 
structive form  of  arthropathy,  since  there  is  dislocation  of  the  joint. 

Another  point  of  this  case  now  deserves  to  be  remarked  upon,  I 
mean  the  absence  of  pain  and  fébrile  reaction.  Without  forming 
an  absolute  rule,  the  absence  of  those  symptoms  is  almost  constant. 

It  was  necessary  to  make  this  qualification  ;  as,  in  fact,  a  patient 
of  the  Hôtel  Dieu,  who  formed  the  subject  of  an  interesting  clinical 
lecture  delivered  by  M.  Bail,  constitutes  an  exception  to  the  rule. 

1  Prequency  of  the  puise,  without  fever,  is  a  common  thing  in  ataxic 
patients. 


CLINIOAL  FACTS.  53 

Sixth  case. — After  having  noticed,  for  some  days,  tlie  existence 
of  cracking  sounds  in  tlie  left  slioulder,  this  man  observed  the 
appearance,  in  one  night's  time,  of  a  tuméfaction  of  the  entire 
upper  extremity^  of  such  volume  that  this  member  was  nearly 
double  the  size  of  the  other.^  Besides  acute  pain  of  the  afFected 
parts,  a  very  marked  élévation  of  the  température  (40°  C.)  was 
observed. 

I  shall  not  dwell  on  a  great  number  of  other  cases  of  arthropathy, 
which  it  bas  been  my  fortune  to  observe,  in  ataxic  patients.  How- 
ever,  before  closing  this  enumeration,  I  should  mention  to  you,  in 
passing,  that  in  M.  Ball's  patient,  the  development  of  a  zona  was 
noticed,  whilst  the  swelling  still  persisted.  This  complication  is 
well  calculated  to  demonstrate  at  least  the  neuropathic,  if  not  the 
spinal  origin,  of  the  affection. 

III. 
I  shall  confine  myself,  gentlemen,  to  this  summary  exposition, 
which  suffices,  indeed,  to   make  you  familiar  with  the  principal 
aspects  of  the  arthropathy. 

A.  To  sum  up  :  without  appréciable  external  cause,  without 
blow  or  fall,  apart  from  any  traumatic  accident  whatever,  the  local 
affection  appears.  At  this  moment  the  incoordination  is  not  yet 
marked,  the  patients  do  not  fiing  about  their  legs,  in  a  disorderly 
manner.  I  must  insist  on  this  détail,  because  it  answers  an 
objection  made  by  Herr  Yolkmann,  which  has  been  repeated  by 
other  surgeons,  who  refuse  to  see,  in  the  arthropathy  of  ataxic 
patients,  anything  else  than  a  traumatic  arthritis  caused  by  the 
mode  of  locomotion  peculiar  to  thèse  patients. 

Nor  can  you  invoke,  hère,  either  the  influence  of  cold,  or  a 
diathetic  state,  goût,  rheumatism,  &c.,  to  account  for  it  ;  the 
articular  affections  due  to  thèse  causes,  hâve,  moreover,  a  totally 
différent  physiognomy. 

B.  This  arthropathy  is  developed  at  a  hut  slightly  advanced 
period  ofthe  spinal  disease,  and  most  commonly  when  its  sympto- 
matology  is  limited  to  the  lightning  pains.  The  incoordination,  it 
is  true,  does  not  generally  make  its  appearance  when  the  arthropathy 
has  occurred.  Thus  it  has,  as  you  observe,  its  place  marked  for  it 
in  the  regular  succession  of  the  symptoms  of  locomotor  ataxia. 

c.  The  arthropathy  is  produced,  generally,  without  prodromes, 
'  'Revue  photographique  des  Hôpitaux  de  Paris,'  1871,  p.  289, 


54  SYMPTOMS  OF  THE  ARTHROPATHY. 

if  we  except,  however,  those  craching  sounds  which  we  find  men- 
tioned  in  a  certain  number  of  cases  (Buj — ,  Lel — ,  Mén — ,  Sic). 

E.  Most  usually,  the  first  phenomenon  discernible  is  extrême 
tuméfaction  of  the  entire  member,  no  previous  difficulty  in  its 
movements  having  existed.  This  tuméfaction  is  formed — 1°,  bj  a 
considérable  hydarthrosis  ;  2°,  by  an  engorgement  which,  in  the 
majority  of  cases,  présents  a  hard  consisteuce,  and  in  which  the 
ordinary  symptoms  of  œdema  are  not  generally  very  marked. 

This  arthropathy  is  not  commonly  accompanied  by  fever,  or  by 
pains  ;  thèse  symptoms  are  only  exceptionalîy  recorded  on  the 
notes. 

At  the  end  of  some  weeks,  or  of  some  months,  the  swelling  dis- 
appears  and  then  ail  returns  to  the  normal  state  {henig^iant  fomi); 
sometimes,  on  the  contrary,  serions  disorders  remain  in  the  joints, 
crackings,  dislocations,  answering  to  a  wearing  down  of  the  osseous 
surfaces,  and  various  luxations  imalignantform).  In  spite  of  thèse 
profound  lésions,  the  member  affected  by  arthropathy  may  still 
serve  for  préhension,  if  it  be  the  upper  extremity  ;  or  for  walking, 
if  the  hip  and  knee  be  the  articulations  affected.  Naturally,  this 
partial  freedom  of  motion  diminishes  if  the  incoordination  makes 
progress,  or  the  luxation  becomes  exaggerated. 

F.  With  respect  to  the  question  of  frequency,  the  order  of  pré- 
férence begins  with  the  knee,  then  cornes  the  shoulder,  next  the 
elbow,  the  bips,  and  the  wrists.  But  the  small  articulations  are  not 
always  spared>  as  we  might  show  by  quoting  some  examples. 


IV. 

Such,  gentlemen,  is  the  symptomatic  picture  which  the  arthro- 
pathy of  ataxic  patients  usually  présents.  Well,  then,  I  ask 
whether  there  is  anywhere  to  be  found,  in  the  domain  of  pathology, 
an  affection  which  corresponds  exactly  to  this  description. 

This  is  a  matter  of  dry  arthritis,  I  am  told,  whenever  I  défend  the 
autonomy,  or  at  least  the  speciality,  of  the  arthropathy  of  ataxia  ; 
and  it  is  six  years  since  I  undertook  this  defence.  I  do  not  dispute 
that  dry  arthritis  is  in  question  ;  but,  by  its  clinical  characters,  I 
maintain  that  this  dry  arthritis  is  quite  spécial,  and  does  not  fall 
under  the  common  law.  Eather  let  us  see  what  we  shall  be  told 
by  a  comparison  of  the  symptomatology  of  âtaxic  arthropathy,  such 
as  I  hâve  sketched  for  you  after  nature,  with  the  classic  description 


DIFFERENTIAL  DIAGNOSIS.  55 

of  dry  arthritis,  the  substance  of  which  I  sliall  borrow  from  a  récent 
and  justly-esteemed  work  J 

1°.  Rarely,  we  are  told,  does  the  quantity  of  fluid  augment  in 
the  joint  affected  by  dry  arthritis.  Now,  this  augmentation  of  fluid 
appears  to  be  constant  in  our  arthropathy. 

2°.  Sometimes,  it  is  true — that  is  to  say,  as  an  exception  to  the 
foregoing  rule — the  effusion  is  considérable,  and  extends  beyond  the 
joint  ;  now,  what  is  tlie  exception  in  dry  arthritis  is,  on  the  con- 
trary,  the  rule  in  ataxic  arthropathy. 

3°.  The  dislocation  of  the  joint,  which  is  very  rare  in  dry 
arthritis,  is  very  fréquent  in  locomotor  ataxia. 

4°.  ïhe  hip-joint  is  principally  affected  in  dry  arthritis  ;  the 
shoulder-joint  takes  only  third  or  fourth  place;  in  ataxic  arthro- 
pathy the  knee  and  then  the  shoulder  are  preferably  attacked,  whilst 
the  hip-joint  is  only  third  or  fourth  in  order. 

5°.  The  course  of  dry  arthritis  is  necessarily  progressive;  it 
never  rétrogrades.  Well,  ataxic  arthropathy  may  rétrograde,  and 
even  be  cured,  when  the  organic  disorders  hâve  not  advanced  too 
far,  as  has  been  demonstrated  by  some  of  the  examples  which  I  hâve 
mentioned. 

6°.  We  are  told  that  in  the  immense  majority  of  cases  the  mono- 
articular  form  of  dry  arthritis  has  been  caused  by  a  traumatic  lésion 
— an  intra-articular  fracture.  I  believe,  indeed,  that  the  fact  is  so. 
But  our  spinal  dry  arthritis,  which  is  often  mono-articular,  acknow- 
ledges  no  such  cause  ;  it  is  not  possible  to  appeal  hère  to  a  trau- 
matic injury,  nor  to  an  intra-articular  fracture. 

7°.  Finally,  let  us  add,  as  a  last  characteristic,  that  the  first 
symptoms  of  ataxic  arthropathy  appear  suddenly  and  unexpectedly, 
whilst  in  dry  arthritis  (still  according  to  the  standard  description) 
the  symptoms  manifest  themselves,  and  become  aggravated,  in  a 
slow  and  progressive  manner. 

Thèse  différences  noted,  I  am  the  first  to  acknowledge  the  analogy 
existing  as  regards  the  cracking  sounds,  the  osseous  tuméfactions, 
&c.  But  I  désire  also  to  demonstrate  what  spécial  features  are  ta 
be  found  in  the  évolution,  the  concaténation,  and  the  character  of 
the  symptoms  ;  in  short,  to  bring  into  the  foreground  the  indubi- 
table relationship  which  exists  between  our  arthritis  and  the  spinal 
affection — a  relationship  which  présents  this  noteworthy  character  in 
particular,  that  the  articular  affection  supervenes  at  a  well-deter- 
^  Follin  et  S.  Duplay,  '  Traite  élémentaire  de  Pathologie  externe,'  t.  iii,  p.  26. 


i6 


PATHOLOGICAL  ANATOMY. 


mined  epocli  iii  tlie  évolution  of  the  spinal  disease.  Let  us  accept 
the  analogies^  but  let  us  not  forget  the  very  striking  diiïerences 
which  are  made  manifest  by  the  comparison. 

V. 

It  is  proper  iiow,  gentlemen,  to  examine  what  information  is 
supplied  us  by  pathological  anatomy.  Undoubtedly,  in  cases  ofold 
standing,  when  the  articular  surfaces,  worn  and  deprived  of  carti- 
lage, hâve  continued  to  move  on  each  other,  the  limbs  bcing  still 
made  use  of  more  or  less  imperfectly,  the  signs  observed  arc  those 
of  dry  arthritis  :  to  wit,  eburnation  and  déformation  of  the  articular 
surfaces,  déformation  of  the  osseous  extremities,  bony  burrs  and 
stalactites,  foreign  bodies,  &c. 

There  are,  however,  two  points  to  which  I  must  request  your 
attention  : 

1°.  The  prédominance  of  wearing  away  over  the  production  of 
bony  burrs  in  récent  cases.  Compare,  for  instance,  the  humérus 
which  I  show  you  (fig.  6),  and  which  cornes  from  an  ataxic  patient, 


PiG.  6. — Superior  extremity  of  a  healtliy  humcrus  and  of  a  humérus  offering 
the  lésions  of  ataxic  artiirojmtliy. 

who  succumbed  two  months  after  the  commencement  of  the  arthro- 
pathy,  with  the  plate  of  Adams's  work,  representing  the  lésions  of 
scapulo-humeral  dry  arthritis,  and  you  will  comprehend  what 
reasons  I  hâve  for  insisting  on  this. 

2°.  I  shall  mention,  in  the  second  place,  the  frequency  of  true 
luxations,  which  are,  to  some  extent,  the  rule  in  ataxic  arthropathy, 
when  the  articulations  admit  such  displacement — in  the  shoulder. 


ATAXIC  AETHROPATHY  :    ITS  SPECIAL  CHARACTER.  57 

for  instance — while  tliey  are  only  exceptions  in  common  dry 
arthritis,  in  whicli  they  are  usually  apparent,  and  net  real. 

On  the  whole,  anatomically  speaking,  our  arthropathy  is  included 
within  the  group  of  dry  arthritis.  To  that  I  hâve  no  serions  ob- 
jection to  make  ;  but  will  it  be  asserted  that  this  anatomo-patho- 
logical  classification  advances  the  matter  much?  If  before  your 
eyes  were  placed,  as  an  anatomical  spécimen,  an  articulation  pre- 
senting  ail  the  signs  of  arthritis  with  sero-fibrinous  or  purulent 
€xudation,  you  would  immediately  indicate  it  by  the  dénomination 
Gipumlent  arthritis,  which  I  hâve  just  employed,  and  histology 
would  change  nothing  in  this  altogether  local  and  anatomical  dia- 
gnosis.  But  vpould  this  completely  résolve  the  problem?  Evi- 
dently  not,  for  the  arthritis  might  hâve  been,  during  life,  one  of  the 
symptoms  of  rheumatism,  of  scarlatina,  of  glanders,  or  of  purulent 
infection  (pysemia),  &c. 

The  same  thing  holds  good,  gentlemen,  with  référence  to  dry 
arthritis,  which  is  often  only  a  manifestation  of  nodose  rheumatism, 
of  tophaceous  goût,  or  one  of  the  lésions  of  Heberden's  disease  ;  as 
it  may  also  be,  finally,  in  some  cases,  the  resuit  of  a  quite  external 
traumatic  cause. 

An  anatomical  examination,  even  with  the  assistance  of  the  most 
advanced  histology,  cannot  supply  everything  ;  its  rôle  is  great,  in 
truth,  but  not  predominating,  and,  in  pathology,  to  notice  this  only 
aspect  is  to  commit  that  error  in  reasoning  which,  in  the  scholastic 
language  of  philosophy,  is  called  an  incomplète  enumeration. 

YI. 

Notwithstanding  the  anatomical  characters  which  connect  it  more 
or  less  closely  with  the  standard  type  of  dry  arthritis,  the  arthro- 
pathy of  ataxia  remains  no  less  a  distinct  variety,  because  both  of 
the  originality  of  the  symptomatic  group  which  it  présents,  and  of 
its  évident  relationship  with  locomotor  ataxia,  of  which,  as  an 
epiphenomenon,  it  really  forms  a  part. 

We  bave  now  to  seek  for  enlightenment  as  to  the  cause  of  this 
relationship.  If  the  solution  of  this  problem  be  difficult,  it  is  not 
impossible,  I  think,  to  supply  it,  at  least  partially. 

In  the  first  place,  I  would  utilise  our  knowledge  of  the  fact  that 
the  existence  of  joint-affections,  more  or  less  subordinated  to  a 
protopathic  lésion  of  the  nervous  System,  is  not,  by  any  means,  an 
exceptional  phenomenon.     I  indicated  this,  wlien  I  told  you  that 


58  PATHOGENY. 

in   my  opinion,  the  arthropathy  of  tabetic  patients  constitutes  a 
genus  in  the  class  of  spinal  arthropathies. 

A.  Again,  we  are  to-day  well  acquainted  with  the  articulai  affec- 
tions which  resuit  from  lésions  of  the  peripheral  nerves  in  the  same 
way  as  herpès,  glossy  skin,  rapid  muscular  atrophy,  and  so  many 
other  trop  hic  disorders  of  the  same  kind.  The  observations  relatiug 
to  wounds  received  in  battle,  which  were  noted  by  Dr.  Weir 
Mitchell,  during  the  American  war,  and  published  anew  in  a  récent 
workji  are  very  instructive,  in  this  respect. 

B.  You  are  acquainted,  likewise,  with  those  singular  articular 
afiPections  which  become  developed  in  limbs  smitten  with  hemi- 
plegia,  owing  to  hsemorrhage  or  ramollissement  of  the  brain,  at  a 
certain  period  of  the  disease,  and  which  come  anatomically  under 
the  description  of  acute  or  subacute  arthritis. 

c.  But,  to  speak  only  of  what  specially  concerns  the  spinal  cord, 
I  believe  I  can  déclare  that  there  is,  perhaps,  not  one  of  the 
morbid  forms  to  which  it  is  subject  which  may  not  provoke,  in 
certain  circumstances,  an  articular  affection  manifestly  correlated 
as  a  symptom  of  the  lésion  of  this  department  of  the  nervous 
centres. 

We  observe  thèse  arthropathies  especially  :  — 1°,  in  paraplegia  from 
Pott's  disease  ;  3°,  in  acute  myelitis  -,  3°,  in  certain  cases  of  tumours 
primarily  occupying  the  spinal  grey  substance  (Gull)  ;  4°,  in  certain 
cases  of  lésions  of  the  grey  substance  determining  progressive 
muscular  atroplïy  (Rosenthal,Remak,  Patruban)  ;  5°,  but  the  case  in 
which  it  is  most  easy  to  demonstrate  the  connexion  which  exists, 
according  to  my  opinion,  between  the  spinal  lésion  and  the  articular 
affection  is  that  of  traumatic  lésions  affecting  the  spinal  cord.  I  shall 
confine  myself  to  tVï^o  examples  in  support  of  what  I  hâve  stated. 

In  a  case,  related  by  M.  Vigucs,  there  was  a  wound  of  the  left 
latéral  half  of  the  spinal  cord  caused  by  a  blow  with  a  sword.  Left 
hemiparaplegia  followed,  with  préservation  of  sensibility  on  the  side 
affected.  About  the  twelfth  day,  tuméfaction  of  the  entire  left 
extremity  was  observed,  and  then  an  arthropathy  of  the  left  knee. 
Pinally,  two  days  later,  a  bed-sore  made  its  appearance  over  the 
right  latéral  portion  of  the  sacrum  and  nates.^ 

'  S.  Weir  Mitchell,  '  Injuries  of  Nerves  and  tlieir  Conséquences,'  Phila- 
delphia,  1872. 

^  For  further  détails,  see  Charcot,  '  Lectures  on  JDiseases  of  the  Nervous 
System/  pp.  79  ei  seq,  New  Sydenham  Society. 


PATHOGENT.  59* 

Thèse  phenomena  might  be  considered  as  only  constituting  a 
simple  coincidence  if  they  were  not  ail  seen  to  be  reproduced,  with 
admirable  regularity,  in  other  analogous  cases.  Such,  in  particular, 
is  that  observed  by  MM.  Joffroy  and  Salmon,  of  whicli  the  follow- 
ing  is  a  summary. 

A  man  was  stabbed  with  a  poignard  which  wounded  the  left 
latéral  half  of  the  cord.  A  few  days  afterwards  there  supervened, 
in  succession,  complète  motor  paralysis  of  the  left  inferior  extremity  ; 
diminution  of  electric  contractility  in  ail  the  muscles  of  this  limb, 
indicating  rapid  and  profound  nutritive  suffering  ;  eschars  occupying 
the  right  nates  [i.  e.  of  the  side  not  paralysed  as  regards  motion), 
although  the  patient  lay  fairly  on  his  back  ;  finally,  an  arthropatjiy 
of  the  left  knee,  in  ail  things  similar  to  that  of  M.  Viguès' 
patient.^ 

Thus,  without  any  appréciable  determining  cause,  there  was  hère 
produced  an  articular  affection  of  an  acute  type  appearing  constantly 
a  few  days  merely  after  the  spinal  lésion,  and  accompanied  by  other 
trophic  disorders,  such  as  bed-sores,  modifications  of  the  electric 
properties  of  the  muscles — trophic  disorders  which  had  ail  evidently 
the  same  origin.  Hâve  we  not,  then,  gentlemen,  suffîcient  éléments 
to  establish  that  the  cord  is,  under  thèse  circumstances,  the  great 
motor  agent  of  the  symptoms  ? 

The  ariliropathy  of  ataxic patients  would  be,  in  the  chronic  state, 
the  représentation  of  the  articular  affections  of  an  acute  type  which 
show  themselves  after  acute  or  subacute  spinal  lésions. 

VII. 

We  should  now  pause  to  seek  what  may  be  the  mechanism  which 
présides  over  the  development  of  thèse  arthropathies,  and  what,  in 
particular,  is  the  région  of  the  spinal  cord  the  altération  of  which 
détermines  the  articular  lésion  ;  for,  manifestly,  ail  the  régions  of 
the  spinal  centre  cannot  be  indiscriminately  arraigned.  Eeverting 
to  locomotor  ataxia,  where  this  question  has  been  especially  studied, 
it  is  clear  that,  a  priori,  the  arthropathy  could  not  be  referred  to  the 
common  and  trite  lésion  of  the  posterior  columns.  We  must  look 
elsewhere. 

By  analogy  with  what  takes  place  in  muscular  atrophy,  and  in 
infantile  paralysis  where  the  muscular  trophic  lésion  is  evidently 
linked  with  an  altération  of  the  anterior  cornua  of  the  grey  sub- 
^  Charcot,  loc.  cit.,  p.  85, 


'60       LESION  OP  CELLS  OF  ANTERIOR  CORNUA. 

stance^  I  had  supposed  that  this  same  région  of  the  grey  substance 
might  also  be  the  starting-point  of  our  articulai-  lésion.  One  fact, 
observed  witli  M.  Joffroy,  may  be  adduced  in  support  of  this  suppo- 
sition. The  same  may  be  said  as  regards  two  other  cases,  studied 
with  MM.  Pierret  and  Gombault.  I  should  add  that  a  certain 
■degree  of  muscular  atrophy  in  the  affected  member  is  frequently 
remarked,  as  a  concomitant  symptom  of  the  arthropathy,  another 
circumstance  which  seems  to  designate  the  anterior  cornua  as  the 
particular  seat  of  the  spinal  lésion.  I  feel,  however,  bound  to  add 
that,  in  a  quite  récent  case  of  tabetic  arthropathy,  in  spite  of 
patient  investigations,  no  atro])hy  of  the  anterior  cornua  on  the  side 
corresponding  with  the  articular  affection  was  to  be  discerned,  on  a 
level  with  the  points  where  it  had  been  found  in  the  previous  cases. 
On  the  other  hand,  in  this  case,  the  spinal  ganglia  were  very 
voluminous,  being  evidently  altered.  It  might  be,  therefore,  that 
they  were  called  upon  to  play  a  part  in  the  production  of  thèse 
arthropathies.  As  regards  the  perii)heral  nerves,  it  was  ascertained 
that  in  the  latter,  as  in  the  former  cases,  no  appréciable  altération 
was  presented  by  thôm.  On  the  whole,  the  question  relative  to  the 
précise  seat  of  the  spinal  lésion  remains  yet  to  be  decided,  and 
requires  new  investigations.  However,  the  subordination  of  the 
articular  to  the  spinal  affection  remains  not  the  less  well  established, 
I  believe,  by  the  weight  of  the  évidence  which  I  hâve  just  laid 
before  you. 

Notwithstanding  the  desideratum  which  I  hâve  noticed  above, 
I  would  recommend,  gentlemen,  to  your  close  attention,  the  arthro- 
pathy of  ataxia  as  a  pathological  and  clinical  fact  of  genuine  worth. 
As  regards  the  first  point,  we  hâve  hère  an  élément  for  the  solution 
of  an  interesting  problem  of  pathological  physiology.  Clinically,  you 
will  learn  to  know  an  affection  which,  if  you  take  up  the  right 
point  of  view,  may  contribute  to  elucidate  the  diagnosis  and  to 
avoid  déplorable  errors.  How  often  hâve  not  I  seen  persons,  not 
yet  familiar  with  this  arthropathy,  misunderstand  its  real  nature, 
and,  whoUy  preoccupied  with  the  local  affection,  even  absolutely 
forget  that  behind  the  disease  of  the  joint  there  was  a  disease 
far  more  important  in  character,  and  which  in  reality  dominated 
the  situation — sclerosis  of  the  posterior  columns. 

I  hâve  donc,  gentlemen,  with  the  considérations  which  I  desired 
to  lay  before  you  in  référence  to  locomotor  ataxia.  My  intention 
could  not  be  to  relate  the  complète  history  of  this  affection  ;  I 


DESIDEEATUM.  61 

have  always  wished  to  treat  the  subject  episodically,  attacking  the 
inost  significant  and  least  known  points.  Those  of  y  ou  who  are 
not  already  versed  in  the  knowledge  of  nervous  diseases  may,  how- 
ever,  recompose  this  classic  picture  by  referring  to  the  numerous 
descriptions  which  have  been  made  in  thèse  latter  times,  and  I  cau- 
not  too  earnestly  recommend  them  to  recur  frequently  to  that  given 
by  Dr.  Duchenne  (de  Boulogne )j  for,  after  ail,  it  remains  ever  the 
best. 

There  is  one  point  which  I  should,  nevertheless,  have  wished  to 
develop  a  little,  if  time  permitted.  I  allude  to  the  question 
of  treatment.  But,  at  this  hour,  there  is  in  that  domain  but  one 
really  new  aspect  worthy  of  detaining  us.  I  mean  the  therapeutical 
application  of  continuons  electric  currents.  This  is  a  subject 
ail  the  more  deserving  of  your  interest,  because  the  application 
of  continuous  currents  is  recommended  not  alone  in  the  treatment 
of  ataxia,  but  also  in  that  of  many  other  chronic  diseases  of  the 
spinal  cord.  Marvels  are  related  with  respect  to  this  method 
beyond  the  Ehine.  What  are  we  to  think  of  thèse  accounts? 
As  yet  I  know  not,  for  to  appreciate  them  at  their  just  value,  one 
should  be  specially  versed  in  thèse  matters.  Happily,  a  favorable 
opportunity  is  offered  to  us.  M.  Onimus,  who,  being  indisput- 
ably  compétent,  has  with  great  zeal  devoted  himself  during  a  year 
in  this  hospital  to  galvanic  electro-therapeutical  investigations, 
has  been  good  enough  to  promise  that  on  next  Saturday,  at  our 
usual  time  of  meeting,  he  will  take  my  place,  and  in  my  stead  will 
indicate  in  a  lecture,  in  a  différent  manner  from  what  I  could,  the 
principles  which  should  control  the  use  of  this  agent.  I  cannot  too 
earnestly,  gentlemen,  invite  you  to  come  to  hear  him.^  In  eight 
days,  I  shall  résume  the  course  of  my  conférences,  when  I  shall 
treat  of  the  symptoms  which  resuit  from  slow  compression  of  the 
spinal  cord. 

>  The  lecture  of  M.  Onimus  was  published  in  the  '  Revue  photographique 
des  Hôpitaux  de  Paris,'  1872. 


PART    SECOND. 


ON   SLOW  COMPRESSION   OF   THE   SPINAL 

CORD. 


LECTURE  V. 

ON  SLOW  COMPRESSION  OF  SPINAL  CORD.    ANATOMiCAL 

PREFACE. 

SuMMARY. — Slow  compression  of  cord  due  to  varions  causes:  im- 
portance  of  its  stiuly. 

Causes  of  compression. — Tumours  of  the  cord  ;  glioma, 
tuiercle,  sarcoma,  carcinoma,  gumma,  hystic  dilatation  of  the 
cord. 

Tumours  primarily  developed  in  the  méninges. — Sarcoma^ 
psammoma,  echinococci,  inflammatory  neoplasias  (internai 
pachymeningitis^  hypertrophie  pachymeningitis). 

Morbid  productio?is  formed  in  the  cellulo-adipose  tissue  of 
the  rachis. — Carcinoma,  sarcoma,  hydatic  hysts,  ahscess. 

Ferteiral  lésions. — Syphilitic  hyperostoses,  dry  arthniis, 
Potfs  disease  :  mechanism  of  compression  of  the  cord.  Can- 
cerous  vertébral  disease.  Painfiùl  paraplegia  of  cancerous 
patients. 

I. 

Gentlemen^ — There  exists  a  peculiar  form  of  paraplegia  whicli 
results  from  the  slow  compression  that  the  spinal  cord  may  suffer 
in  varions  points  of  its  course  within  the  rachidian  canal.  The 
organic  lésions  which  are  capable  of  leading  to  this  resuit  are  very 
différent  in  kind.  Thus^  for  instance,  we  find  inflammatory  neo- 
plasms,  cancerous^  sarcomatous,  or  tuberculous  tumours,  syphilitic 
products,  even  parasitical  growths,  &c. 

Clinically  considered,  thèse  lésions,  howsoever  varied  as  regards 
their  origin,  should  be  brought  together  at  least  for  an  instant. 
For,  indeed,  the  phenomena  which  they  occasion  by  interrupting 
the  course  of  the  nerve-fibres  in  the  cord,  often  constitute  the  fîrst 
symptoms  which  strike  the  attention  of  the  physician,  and,  given  a 

VOL.  II.  5 


66  SLOW  COMPRESSION  0¥  THE  SPINAL  CORD. 

paralysis,  it  becomes  a  question   for  liim  to  trace  it  up  to  the 
organic  cause  whicli  provoked  it. 

It  is  to  this  kind  of  paraplegia  that  I  purpose  consecrating  our 
conférences  of  this  and  the  following  days.  However,  before 
describing  to  you  tlie  particular  symptoms  whicli  reveal  its  présence 
during  life,  it  seems  to  me  indispensable  to  compare  together  the 
œany  organic  lésions  which  occasion  it,  as  regards  their  patho- 
logical  anatomy  and  physiology.  Tor,  gentlemen,  the  effects  of 
slow  compression  on  the  spinal  nerve-centre  vary  but  little,  save 
as  respects  the  région  of  the  centre  involved,  and,  apart  from  this 
circumstance,  they  always  show  themselves  nearly  identical,  what- 
ever  may  be  the  cause  which  determined  the  compression.  Hence, 
it  is  not  in  this  direction  that  we  should  generally  seek  for  the 
révélation  of  distinguishing  signs. 

But,  on  the  one  hand,  before  they  begin  to  alFect  the  cord  in  the 
natural  course  of  their  évolution,  and,  on  the  other,  in  the  very 
time  when  they  détermine  a  more  or  less  effective  compression  of 
this  organ,  the  lésions  in  question  often  hâve  an  anatomical  and 
clinical  history  peculiar  to  themselves.  Now,  it  is  this  history, 
above  ail,  which  it  will  be  necessary  to  consult  in  order  to  find 
the  characters  which  permit  us  to  ascend  to  the  source  of  the 
disease. 

II. 

This  préface,  gentlemen,  dispenses  with  the  necessity  of  dwelling 
on  the  subject  at  length  in  order  to  set  prominently  before  you 
the  importance  of  the  study  to  which  we  are  about  to  dévote  our- 
selves.  Let  it  suffice  for  me  to  remind  you  that  a  considérable 
number  of  the  organic  lésions  which  we  are  about  to  pass  in  review 
are  most  common,  and  consequently  hold  the  foremost  rank  in  or- 
dinary  clinical  work.  Vertébral  caries  or  Pott's  disease,  vertébral 
cancer,  intra-rachidian  tumours,  may,  in  fact,  be  mentioned 
as  amongst  the  most  fréquent  causes  of  paraplegia  by  slow 
compression. 

III. 

With  a  view  to  establish  a  little  order  in  the  enumeration  which 
is  to  follow,  we  will  group  the  lésions  in  question  after  the  position 
they  occupy  at  the  outset  of  their  development.  With  this  aim,  it 
is  proper  to  recall  to  your  memory,  in  a  rapid  way,  the  principal 


CAUSES  OP  COMPEESSIOîf.  67 

dispositions  whicli  are  presented  by  the  cord  iu  tlie  midst  of  tlie 
spinal  canal.  You  are  aware  that  this  nervous  column,  covered 
witk  its  proper  envelope,  the  pia  mater,  is,  as  it  were,  suspended  iu 
a  sort  of  sheatli  formed  bj  the  dura  mater,  to  which  it  is  scarcely 
attached,  except  by  the  médium  of  the  nerve  roots  and  dentated 
ligament.  The  arachuoid  plays  the  part  of  a  serous  membrane  in- 
terposed  between  the  pia  mater  and  the  dura  mater.  The  latter, 
like  the  cord,  is  itself  suspended  in  the  spinal  canal, — an  osseous 
canal  more  or  less  flexible  aceording  to  the  région.  The  dura 
mater  touches  this  canal  nowhere,  unless  at  the  conjugation  notches, 
which  serve  to  let  the  nerves  pass  through,  and  again  in  the  cervical 
région  at  a  point  which  corresponds  to  the  anterior  face  of  the 
spinal  dura  mater.  I  should  add  that  this  contact  is  altogether 
indirect,  médiate,  and  effected  solely  by  means  of  hgamentous  tracts. 
Everywhere  else  the  dura  mater  is  separated  from  the  osseous 
parietes  by  a  layer  of  adipose  tissue,  which  gives  passage  to  the 
arteries,  veins,  and  nerves.  Finally,  gentlemen,  the  rachidian  canal 
encloses  the  cord  and  its  envelopes  in  a  well-nigh  hermetic  manner  ; 
except,  however,  at  the  inter-vertebral  notches,  which  may,  as  we 
shall  see,  admit  certain  pathological  products  formed  outside  of  this 
osseous  conduit. 

I  hâve  been  obliged  to  remind  you,  thus  summarily,  of  the  topo- 
graphical  anatomy,  because  there  is  probably  not  one  of  the  parts 
I  hâve  just  enumerated  that  may  not  become  the  seat  of  a  morbid 
product,  which,  by  the  ulterior  progress  of  its  évolution,  may  come 
into  contact  with  the  cord,  and  exert  a  more  or  less  well-marked 
compression  upon  it. 

It  foUows  from  this  :  i°,  that  products  which  hâve  begun  their 
career  outside  of  the  rachidian  canal  may  intrude  themselves  into 
this  canal  through  the  intervertébral  notches  ;  2°,  that  others  may 
develop  :  a,  in  the  bones,  or  periosteum  ;  b,  in  the  cellulo-adipose 
tissue  external  to  the  dura  mater  (peri-meningeal)  ;  c,  at  the 
expense  of  the  roots  and  nerve  trunks  ;  d,  in  the  dura  mater  or  in 
the  arachnoid  and  the  pia  mater  ;  e,  finally,  in  the  cord  itself. 

IV. 

Let  us  consider,  in  the  first  place,  the  morbid  growths  whicb 
originate  iu  the  spinal  cord  itself.  Hère,  gentlemen,  we  hâve  of  its 
kind  a  group  truly  separate  and  distinct,  for  the  mechanism  whereby 
they  engender  paralysis  cannot  be  assimilated,  without  reserve,  to 


68  INTEA-SPINAL  TUMOURS.      GLIOMA. 

that  whicli  is  callecl  into  action  by  compression  from  without 
iuwards.  In  fact,  generally  speaking,  the  neoplasms  wliich  compose 
tlie  tumours  slowly  substitute  themselves  for  the  nerve-elements, 
rather  tlian  mechanically  compress  them.  Again^  you  easily  un- 
derstand  that  the  efFects  caused  by  the  présence  of  thèse  tumours 
necessarily  manifest  themselves _//w;ï  tJie  outset,  by  symptoms  cor- 
responding  to  the  interruption  of  spinal  nerve-fibres  ;  whilst  in  the 
case  of  morbid  growths,  formed  externally  to  the  cord,  thèse  phe- 
nomena  only  show  themselves  in  a  tardy  manner,  whence  you  hâve 
a  first  distinguishing  character,  whicli  I  content  myself  with  noticing 
now.     Its  application  will  be  found  hereafter. 

Intra-spinal  tumows.-^We  shall  confine  ourselves,  as  regards 
thèse  tumours,  to  a  brief  enumeration,  because,  on  the  whole,  they 
are  rather  rare. 

a.  The  glionia  comes  foremost;  not,  indeed,  on  account  of  its 
frequency,  for  it  is  an  exceptional  lésion,  but  because  we  hâve  hère 
a  product  spécial  to  the  région,  so  to  speak.  In  fact,  the  organs  in 
which  gliomata  hâve  been  hitherto  especially  found  are  the  brain 
and  spinal  cord. 

Every  tumour,  you  know,  according  to  Mûller's  law,  has  its 
paradigm — its  physiological  type.  Now,  hère,  the  neuroglia  is  the 
normal  tissue  at  whose  expense  is  developed  the  tumour,  denominated 
glioma,  and  whose  characters  are  reproduced  by  it. 

It  présents  itself  under  the  appearance  of  soft,  greyish  masses, 
which,  by  their  colour  and  other  characteristics,  recall  the  grey 
substance  of  the  nerve-centres,  in  which,  indeed,  they  form  by 
préférence.  The  gliomata  are  not  well  defined,  easily  enucleated 
tumours  ;  they  fuse  by  imperceptible  shades  with  the  nerve-tissue. 
Nevertheless,  the  glioma  is  a  tumour  in  the  common  acceptation  of 
the  Word,  and  this  is  a  feature  which  distinguishes  it  from  foci 
of  sclerosis,  with  which  it  has  so  many  analogies,  for  the  parts 
invaded  by  it  (the  optic  thalami  in  the  brain,  for  instance)  become 
swollen  in  a  remarkable  manner,  whilst  still  preserving  their 
form. 

Histologically,  we  find  in  thèse  tumours  characters  which  recall 
sclerosis,  for  they  are  almost  exclusively  constituted  by  numerous 
cellular  and  nucleate  éléments,  which  are  nothing  else  than  myelocites 
immersed  in  a  finely  granular  amorphous  substance.  Under  the 
influence  of  chromic  acid,  this  intermediate  amorphous  substance  is 
decomposed,  if  we  may  use  the  expression,  into  an  infinity  of  slender 


TUBERCLE,  GUMMA,  ETC.  69 

fibrilsj  presenting  a  great  resemblance  to  what  is  seen  in  confirmed 
sclerosis.  But  tlie  fundamental  différence  lies  hère  :  nowliere  can 
we  detect  the  alveolar  disposition  proper  to  the  reticulum  of  the 
neuroglia,  and,  moreover,  the  nerve-elements  are  completely 
absent. 

I  shall  not  dwell  longer  on  the  glioma  for,  as  regards  the  cord, 
its  potency,  at  présent,  is  limited  to  three  or  four  more  or  less 
imperfect  cases.  I  cannot,  however,  abstain  from  mentioning  the 
foUowing  peculiarity  :  the  gHoma  is  a  very  vascular  growth,  and 
the  vessels  which  traverse  it  are  specially  liable  to  burst,  whence 
effusions  of  blood,  of  varions  extent,  within  the  core  of  the  tumour. 
Thèse  hœmorrhages  may  reveal  themselves  during  life  by  sudden 
symptoms,  and,  after  death,  may  mislead  by  suggesting  the  notion 
that  the  lésion  was  a  primary  hsematomyelia  (or  cord-hsemorrhage), 
a  rare  affection,  the  reality  of  which  lias  even  been  questioned. 

i.  After  the  glioma  I  would  mention  solUary  tubercle,  which  may 
be  regarded  as  one  of  the  most  fréquent  of  intra-spinal  tumours. 
It  generaUy  coïncides  with  the  existence-  of  tubercles  developed  in 
other  organs. 

c.  The  différent  varieties  of  sarcoma  and  carcînoma  can  scarcely 
be  said  to  show  themselves,  at  the  first  onset,  in  the  cord  itself. 

d.  The  gumma  or  sr/pJiiloma  is  also  a  morbid  growth,  but  little 
fréquent  as  an  intra-spinal  tumour.  There  exist  in  the  records  of 
our  science  about  three  or  four  examples  of  tliis  kind,  and  "the 
description  is  mostly  insufïicient.  Nevertheless,  I  hope  to  show 
you,  gentlemen,  in  connexion  with  a  case  which  bas  recently  fallen 
under  my  observation  that  we  may,  with  the  help  of  circumstances, 
recognize  with  some  précision  during  life  the  présence  of  a  syphilitic 
lésion  in  the  cord. 

e.  I  do  not  wish  to  quit  the  group  of  intra-spinal  tumours 
without  pointing  out  to  your  notice  the  Jcysiic  dilatation  which  is 
sometimes  presented  by  the  central  canal  of  the  spinal  cord.  In  a 
case  of  GuU's,  and  in  some  others,  this  lésion  was  indicated,  during 
life,  by  paresis  with  muscular  atrophy  of  the  upper  extremities. 
The  expansion  in  this  case,  affected  the  canal,  throughout  nearly  its 
whole  length,  in  the  cervical  enlargement.  The  last-mentioned 
symptom,  muscular  wasting,  is  easily  understood,  because  in 
expanding,  the  dilated  canal  should  almost  infallibly  détermine  a 
more  or  less  strong  compression  of  the  anterior  cornua  of  tlie  grey 
substance. 


70  TUMOURS  OP  DURA  MATER, 


Y. 


Tumours  pnmanly  developed  in  the  méninges. — Gentlemen,  it 
may  be  said  with  Dr.  Gull  that  the  majority  of  tumours 
"whicli  are  developed  primarily  in  the  méninges,  belong  to 
the  class  of  benignant  neoplasms.  Carcinoma  does  not  figure 
tliere  except  in  a  merely  accessory  way,  at  least  as  a  primary 
tumour.  It  is  on  the  dura  mater  and  on  its  inner  surface  chiefly 
that  most  of  the  morbid  growths  occur.  They  form  more  or  less 
rounded  tumours,  sessile  or  pedunculated,  which  do  not  commonly 
acquire  a  large  volume.  Their  dimensions  are  mostly  those  of  a 
bean,  a  cherry,  or  at  the  very  most  of  a  small  Q2,g. 
We  shall  mention  amongst  the  most  common  : 
1°.  The  différent  varieties  of  sarcoma,  comprising  the  fuso- 
cellular  sarcoma,  and  the  medullary  or  round-celled  sarcoma. 

2°.  The  psammoma  or  arenaceons  tumour,  which  deserves  to  hold 
our  attention  a  little,  because  hère  we  hâve  a  growth  proper  to  the 
région  ;  this  is  the  angiolitJdc  sarcoma  of  MM.  Eanvier  and  Cornil. 
The  tumour  is  composed  of  little  rounded  or  muriform  calcareous 
masses,  having  an  envelope  of  cells  adhèrent  each  to  each  which 
by  their  arrangement  remind  us  of  epidermal  globes. 

The  physiological  type  is  represented  by  small  analogous  tumours 
which  exist,  in  the  normal  state,  on  the  surface  of  the  cranial  dura 
mater,  and  of  the  choroid  plexuses.  In  the  cranial  cavity,  their 
volume  is  rarely  considérable  enough  to  détermine  symptoms  of 
compression  ;  but,  in  the  rachidian  canal,  even  when  they  do  not 
attain  the  size  of  a  haricot  bean,  thèse  tumours,  developed  under 
constraint  between  the  dura  mater  and  the  cord,  promptly  occasion 
the  flattening  of  the  nerve  column  and  the  phenomena  which  are 
its  conséquences. 

3°.  I  cannot  dispense  with  a  mention,  as  we  pass,  of  the  existence 
of  ecJdnococci,  developed  between  the  viscéral  layer  of  the  arachnoid 
and  the  pia  mater,  as  différent  cases  hâve  demonstrated,  amongst 
others,  those  related  by  Bartels  and  Esquirol. 

4°.  Finally,  in  référence  to  the  dura  mater,  I  shall  call  your 
attention  to  the  infiammatory  neoplasms  capable  of  giving  rise  to  the 
phenomena  of  slow  spinal  compression,  although,  in  such  circum- 
stances,  there  is  no  longer  question  of  tumours  in  the  rigorous 
acceptation  of  the  term.     Thèse  are  : 


LESIONS  OF  CELLULO-ADIPOSE  TISSUE.  71 

rirst,  internai  ^^achymeningitls  which,  hère  as  in  the  cranium^ 
inay  become  the  starting-point  of  a  hœmatoma  (as  in  the  case  of 
Eilhle).' 

Second,  a  form  of  pachymeningitis  particularly  fréquent  at  the 
cervical  enlargement,  and  which  might  be  termed  hypertropldc,  for 
it  especially  consists  in  an  often  enormous  thickening  of  this  mem- 
brane. Commonly  also  the  other  méninges  participate  in  the 
altération.  The  membranous  canal,  formed  by  the  méninges, 
grows  narrowed,  the  cord  becomes  strangled  as  it  were  by  its 
hypertrophied  envelopes,  which,  themselves,  at  a  given  moment, 
suffer  a  sort  of  retraction  operating  through  a  spécial  mechanism, 
and  which  differs  from  ordinary  compression.  The  affection  which 
produces  thèse  accidents  is  not  rare,  and  it  is  possible  to  identify  it, 
during  life,  by  the  help  of  certain  characters.  Thus  it  merits,  from 
every  point  of  view,  a  spécial  study  which  we  shall  undertake  at  a 
future  opportunity.^ 

VI. 

With  respect  to  the  cellulo-adipose  tissiie  qf  the  rachis,  this  also 
gives  birth  to  morbid  growths  which,  in  developing,  succeed  in 
exerting  médiate  compression  on  the  cord,  by  thrusting  in  the  dura 
mater. 

1  hâve  often  seen  carcinoma  occupying  this  région  in  certain 
cases  of  cancer  of  the  breast;  other  tumours,  and  particularly 
sarcoma  and  hydatid  kysts,  may  take  up  their  primary  position  hère. 
According  to  Traube,  abscesses  are  also  formed  in  this  cellulo- 
adipose  tissue  which,  issuing  out  through  the  intervertébral  notches, 
make  their  appearance  on  the  borders  of  the  rachis.  But,  in 
gênerai,  it  is  the  inverse  of  this  which  meets  the  eye  ;  tumours  of 
différent  kinds,  originating  on  the  outside  but  in  the  vicinity  of  the 
rachis,  advance  towards  the  interior,  naturally  through  the  apertures 
offered  by  thèse  notches,  and  so  penetrate  into  the  spinal  canal. 
Thus  it  was  with  the  hydatid  kysts  described  by  Cruveilhier,  and 
thé  p'e-vertébral  abscesses,  those,  for  instance,  which  are  developed 
behind  the  pharynx  and  which  are  sometimes  designated,  in 
Germany,  by  the  term  Angina  Ludovici,  from  the  name  of  the 
author  who  first  described  them  properly.  At  other  times,  thèse 
products  make  themselves  a  way  by  a  différent  method.  They 
introduce  themselves  into  the  spinal  cavity  by  a  broader,  if  not  a 

1   Rûhle,  '  Greefswalder  Medizinischc  Beitrage,'  I  Bd,,  Dantzig,  1863,  p.  8, 

2  See  Appendix,  note  a. 


72  VERTEBRAL  LESIONS. 

sliorter  patli,  by  disintegrating  and  dissociating  the  vertebrœ.  I 
would  quote,  for  instance,  in  connexion  witli  this  subject,  the  action 
of  hydatids  and  of  aortic  aneurisms. 

Let  us  likewise  mention  the  neuromata,  the  fibromata,  and  the 
myxomata,  developed  at  the  expense  of  the  connective  envelope  of 
the  nerves,  the  structure  of  which  appears  formed  on  the  model  of 
the  mucous  network  of  Wharton^s  gélatine.  Thèse  tumours 
détermine  first  the  compression  of  the  nerve  éléments,  then,  lacking 
room  in  the  spinal  canal,  they  thrust  in  the  dura  mater  and  by  that 
médium  press  upon  the  cord  itself. 

VII. 

Vertébral  lésions. — I  corne,  gentlemen,  to  what  is  undoubtedly 
the  most  important  point  of  this  exposition  : 

1°.  I  shall  not  speak  of  the  syjildlitic  hyperostoses ,  which  hâve 
been  admitted  ralher  on  hypothetical  grounds  than  on  rigorous 
examination,  so  far  at  least  as  regards  their  forming  tumours 
voluminous  enough  to  compress  the  cord. 

2°.  I  shall  also  confine  myself  to  mentioning  dry  arthritis  of 
the  inter-apophysar  vertébral  articulations,  hypertrophy  of  the 
odontoid  process  amongst  others,  which  arthritis  in  certain  rare 
cases,  very  rare  cases  indeed,  since  Adams  who  occupied  himself 
specially  with  this  question  never  met  any  examples,  is  capable  of 
producing  the  phenomena  of  spinal  compression.  By  way  of 
mémorandum,  I  would  cite  the  report  of  a  case  by  M.  Bouchard, 
which  was  noted  in  my  wards. 

3°.  But  I  shall  make  a  spécial  delay  on  coming  to  PoWs  disease 
(vertébral  caries),  and  vertébral  cancer.  Thèse  affections  may,  in 
fact,  be  counted  amongst  the  most  common  causes  of  organic  para- 
plegias,  considered  geuerally,  and  of  paraplegias  by  compression, 
studied  in  particular.  Of  necessity,  I  shall  not  enter  into  ail  the 
détails  which  would  suit  a  complète  history  of  thèse  affections  ;  I 
shall  dévote  myself  exclusively  to  those  points  which  relate  most 
directly  to  the  compression  which  thèse  lésions  hâve  the  power  of 
determining. 

A.  I  will  commence  with  Pott-'s  disease.  It  is  a  remarkable 
thing  that,  though  this  is  evidently  a  very  common  disorder,  people 
are  not  yet  agreed  as  to  the  mauner  in  which  the  spinal  cord  becomes 
alTected.    The  desideratum  has  been  fulfilled,in  récent  days,  by  one 


pott's  disease.  73 

of  my  clinical  assistants,  M.  Micliaud,  in  an  inaugural  disserta- 
tion which  I  recommend  to  your  attention.i 

Generally,  it  is  summarily  stated  that  the  paraplegia  is  a  resulfc, 
in  such  cases,  of  the  exaggerated  and  often  angular  bending  of  the 
rachidian  canal,  when  one  or  several  of  the  vertébrée  hâve  givenway. 
But  then,  as  Boyer  and  Louis  remarked,  the  paraplegia  may  dis- 
appear,  whilst  the  curve  still  continues  to  the  same  degree.  Again, 
paraplegia  from  Pott's  disease  is  to  be  seen  when  there  is  not  the 
slightest  trace  of  deformity.-  Pinally,  a  third  argument  against  the 
current  opinion  lies  in  the  fact  that  we  know,  as  M.  Cruveilhier 
particularly  noted,  that  the  spine  may  présent  the  most  extraordinary 
deformities,  whilst  the  cord  continues  intact. 

According  to  our  researches,  gentlemen,  this  is  how  things 
happen  in  the  immense  majority  of  cases  :  at  the  onset,  it  is  possible 
that  a  caseous  abscess,  formed  at  the  affected  veterbrœ,  may  thrust 
back  the  anterior  vertébral  ligament,  which  then  makes  a  bulge  in 
the  canal  ;  but  this  is  not  the  ordinary  mode.  The  vertébral  liga- 
ment becomes  dissociated,  ulcérâtes,  and,  lastly,  gets  destroyed  at  a 
certain  point  in  such  a  way  that,  as  M.  Michaud  has  well  shown,  the 
pus  of  osseous  origin  cornes  into  contact  with  the  anterior  face  of  the 
dura  mater  which  subsequently  takes  on  inflammation,  after  its  manner. 
A  peculiar  kind  of  external  caseous  pachymeningitis  is  hère  produced, 
whose  modeof  évolution  has  been  minutely  studied  by  M.  Michaud. 
It  is  really  the  external  surface  of  the  dura  mater  which  végétâtes 
and  proliférâtes,  for  the  middle  portion  and  the  internai  surface 
frequently  remain  quite  intact.  The  products  of  the  inflammation 
dissociate  thèse  superficial  layers  and,  preserving  a  certain  degree 
of  cohérence,  go  to  form  a  sort  of  mushroom  of  more  or  less  volume, 
which  is,  in  reality,  the  agent  of  compression.  In  the  core,  the 
embryoplastic  éléments  are  still  perfectly  visible  to  histologie 
scrutiny  ;  on  the  surface  they  hâve  undergone  caseous  metamor- 
phosis.  This  inflammation  extends  along  the  dura  mater  from 
before  backwards,  but  the  mushroom  is  rarely  transformed  into  a 
perfect  ring  or  belt,  so  that  the  cord  only  appears  compressed  on 
one  portion  of  its  external  surface. 

It  need  not  be  added  that  the  nerve-trunks,  in  their  course 
through  the  altered  portions  of  the  dura  mater,  are,  in  their  turn, 
the  seat  of  more  or  less  considérable  lésions  which  will  be  mani- 

^  Michaud,  '  Sur  la  méningite  et  la  myélite  dans  le  mal  vertébral.'  1871. 
-  Cas  de  M,  Liouville. 


74  CANCEROUS  VERTEBEAL  DISEASE. 

fested^  during   life,  by  corresponding   symptoms  ;    this  is  a  fact 
wliicli  we  shall  make  use  of  hereafter. 

B.  Along  with  the  vertébral  disease  of  Pott,  I  will  place  tlie 
canceroîcs  vertehral  disease.  Rarer  thau  Pott's  disease,  vertébral 
cancer  nevertheless  présents  itself  frequently  enough  in  clinical 
practice.  We  meet  it  tolerably  often  at  La  Salpêiriere,  situated 
as  we  are,  indeed,  under  quite  spécial  conditions  of  observation. 

Seldom  primary,  vertébral  cancer  shows  itself  particularly  after 
cancer  of  the  breast,  and  principally  when  tins  takes  on  the  hard 
forms  of  carcinoma.  It  is  likewise  observed  consecutively  on  rénal 
cancer,  on  gastric  cancer,  on  cancerous  degeneration  of  the  pre- 
vertebral  ganglions,  supervening  either  as  a  secondary  manifesta- 
tion or  caused  by  a  sort  of  graduai  extension,  a  direct  propagation, 
as  it  were,  of  the  disease. 

Vertébral  cancer  has  been  the  object  of  important  works  ;  I  would 
cite  amongst  others  those  of  C.  Hawkins,  Leyden,  Cazalis,  my  own 
researches,  and,  finally,  the  thesis  of  M.  L.  Tripier  which  is  founded 
chiefly  on  information  obtained  in  this  hospital.^  Let  us  now  see 
how  vertébral  cancer  appears  on  anatomical  examination. 

There  are  cases  in  which  the  cancerous  nuclei,  developed  in  a 
small  number  in  the  core  of  the  bodies  of  the  vertébrée  remain 
absolutely  latent  ;  this  variety  of  vertébral  cancer  is  very  common, 
but  it  is  not  this  kind  which  should  command  our  interest  in  the 
présent  lecture. 

In  other  instances,  the  bodies  of  the  vertébrée,  infiltrated  through- 
out  by  the  neoplasm,  soften  and  give  way  under  the  weight  of  the 
frame.  This  subsidence  often  takes  place  without  the  supervention 
of  any  well-marked  déviation,  and  this  peculiarity  is  of  some  import- 
ance. In  other  cases,  we  remark  a  rounded  curve,  of  large  radius, 
which  is  very  différent  from  that  of  Pott's  disease. 

Cancerous  infiltration  does  not,  however,  confine  itself  to  the 
bodies  of  the  vertébrée,  it  also  invades  the  laminse  and  the  apophyses. 
In  such  cases,  the  vertébrée  are  occasionally  as  soft  as  caoutchouc. 
One  of  the  most  serions  conséquences  of  this  state  of  things  will  be 
the  compression  of  the  nerve-trunks,  in  less  or  greater  number,  on 
their  passage  through  the  intervertébral  notches,  owing  to  a 
mechanism  which  I  hâve  already  described. 

This   compression,  you  will  carefully  remark,  may  exercise   its 

'  Tripier,  '  Du  cancer  de  la  colonne  vertébrale  et  de  ses  rapports  avec  la 
paraplégie  douleureuse,  1866. 


CANCEEOUS  VERTEBEAL  DISEASE.  75 

influence,  tliougli  the  cord  be  not  involved,  and  in  tliis  way  is  pro- 
duced  that  symptomatic  grouping  wliicli  I  hâve  proposed  to  desig- 
nate  under  the  name  of  painfitl  paraplegta  qf  cancero%s  patients  ; 
for  not  only  are  the  uerves  thus  compressed  by  the  weight  of  the 
vertébral  column,  the  starting-point  of  very  keen  pains,  but  they 
may  also  bring  about  a  weakening  of  muscular  strength  in  the 
merabers  to  which  they  extend — a  weakening  which  is  itself 
followed,  in  the  long  run,  by  a  more  or  less  marked  atrophy  of  the 
muscles. 

Compression  and  nerve-irritation  are  frequently  at  work,  and  that 
to  a  high  degree,  in  vertébral  cancer.  They  exist  also  in  Pott's 
disease,  but  less  marked,  more  circumscribed,  and  operating,  indeed, 
by  another  mechanism.  In  such  circumstances,  the  nerve  irritation 
is  occasioned  by  their  passage  through  the  inflamed  points  of  the 
dura  mater.  Nerve  compression  may  also  be  produced  in  the  case 
of  tumour  or  inflammatory  neoplasm  originating  in  the  méninges  ; 
hence  it  is  an  élément  which  shows  itself,  in  varions  proportions,  in 
ail  cases  of  organic  lésions  which,  developed  externally  to  the  cord, 
produce,  in  one  of  their  phases,  the  compression  of  this  organ. 
The  tumours  alone  which  are  primarily  developed  in  the  central 
parts  of  the  cord  escape  this  law,  and  that  is  a  circumstance  which 
must  be  considered  in  diagnosis. 

But  let  us  return,  gentlemen,  to  vertébral  cancer.  The  com- 
pression of  the  nerve  trunks  by  the  means  which  hâve  just  been 
indicated  is  not  ail.  Commonly,  a  circumstance  is  superadded, 
the  conséquence  of  which  is  to  entail  the  participation  of  the  cord. 
The  cancerous  masses  make  their  way  out  of  the  bodies  of  the 
vertébrée  ;  they  reach  the  periosteum,  and  the  dura  mater,  which, 
for  the  most  part,  présents  an  obstacle  that  is  soon  conquered, 
and  thus  the  cord  becomes  involved.  There  are,  in  truth,  many 
other  combinations,  but  I  think  the  foregoing  explanations  will  be 
sufficient  to  make  you  acquainted  with  the  most  usual  phenomena. 


LECTUEE  VI. 

ON  SLOW  COMPRESSION  OF  THE  SPINAL  CORD.  ANATO- 
MICAL  MODIFICATIONS  IN  CASES  WHICH  TERMINATE  IN 
CURE.  SYMPTOMS.  PSEUDO-NEURALGIAS.  PAINFUL 
PARAPLEGIA  OF  CANCEROUS  PATIENTS. 

SuMMARY. — Anatomical  modifications  îvJiich  tlie  cord  undergoe%  at 
the  compressed point.  Changes  in  form  ;  softeniiig,  induration. 
Interstitial  myelitis.  Ascending  and  descending  consécutive 
sclerosis.  There  may  he  restoration  of  function,  notwithstand- 
ing  tJie  existe?ice  of  pro/ound  lésions.  Régénération  of  nerve 
tubes  at  the  compressed point. 

Symptoms. — Extrinsic  and  intrinsic  symptoms.  Topogra- 
phical  anatomy  of  the  vertébral  région. 

Extrinsic  symptoms  :  pseudo-neuralgias.  Pseudo-neiiralgias 
in  cases  of  rachidian  tumoiirs,  of  Pott's  disease,  and  in  can- 
cerous  vertébral  disease. 

Painfitl  par aplegia  of  cancer  DUS  patients.  Pain,  its  charac- 
ters,  paroxysms.  Tegumentary  hyperœsthesia.  Eruption  of 
zona  along  the  course  of  painfnl  nerves  ;  circumscribed  cuta- 
neous  anœsthesïa  ;  partial  muscular  atrophy  and  contracture. 
Déformation  of  the  vertébral  column.  BifficjiUy  of  diagnosis  in 
certain  cases  :  osteomalacia,  hypertrophie  cervical  pachymenin- 
gitis,  spinal  irritation,  Sfc. 

GentlemeNj — You  know  how  we  passed  in  review,  at  our  last 
meeting,  the  principal  organic  lésions  which  may  détermine  slow 
compression  of  the  spinal  cord.  At  présent,  we  must  study  the 
effects  which  this  compression  produces  on  the  texture  of  the 
cord. 

There  is  no  question  hère,  gentlemen,  of  purely  mechanical 
phenomena  ;  the  cord  reacts  after  its  manner,  and  sooner  or  later 
inflames  under  the  influence  of  compression,  whatever  may  be  its 


ALTEEATlOîf  OF  THE  COUD.  11 

cause.     Tins  is  a  fact  which,  I  liope.  will  be  easily  placed  beyoud  a 
doubt. 

I. 

Let  us  first  examine  wliat  takes  place  on  tlie  spinal  cord  at  the 
point  where  it  is  compressed. 

Possibly  in  the  early  stages  we  had  there  simple  comiiressîon  only, 
without  other  modification  than  what  results  from  pressure  exercised 
on  the  parts.  This^  most  assuredly,  was  what  took  place  in  the 
two  following  cases.  In  the  first^  which  Ehrling  relates,  the  com- 
pression was  due  to  the  luxation  of  a  cervical  vertebra.  An  opéra- 
tion for  its  réduction  was  successful,  and,  at  the  end  of  eight  days, 
ail  symptoms  of  compression  had  disappeared.  The  second  case, 
mentioned  by  M.  Brown-Séquard,  relates  to  a  patient,  sufFering 
from  Pott's  disease,  who  suddenly  exhibited  symptoms  of  compres- 
sion deuoted  by  complète  paraplegia  ;  on  the  application  of  a 
suitable  prothetic  apparatus  ail  trace  of  paralysis  vanished  in  fifty 
hours.  In  both  of  thèse  cases,  rather  exceptional  as  they  are,  the 
compression  came  abruptly  into  action.  Consequently,  they  differ 
from  those  which  should  hère  engage  our  spécial  attention.  The 
numerous  observations  which,  with  M.  Michaud,  we  made  in  the 
course  of  the  last  two  years  relative  either  to  tumours  or  to  Pott's 
disease,  hâve  always  shown,  even  at  an  early  period,  a  more  or  less 
profound  altération  of  texture,  in  addition  to  the  change  of  form 
occasioned  by  the  compression. 

Mention  lias  sometimes  been  made  of  softening  by  ischsemia, 
comparable  to  that  produced  by  arterial  oblitération,  and  super- 
veuing  in  the  spinal  cord  on  a  level  with  the  point  of  compression. 
M.  L.  Tripier  has  called  attention  to  this  secondary  lésion  in  the 
case  of  cancer  of  the  vertébral  column.  This,  however,  is  undoubt- 
edly  rare,  and  I  should  also  add  that  the  anatomical  examination, 
as  conducted  in  the  case  in  question,  is  not  beyond  criticism,  the 
cord  having  only  been  examined  in  the  fresh  state,  and  not  after  a 
hardening  process  which  could  alone  hâve  made  connective  hyper- 
plasias  clearly  manifest. 

In  fact,  according  to  my  observation,  the  naked  eye  perceives 
sometimes  a  softening,  sometimes  an  induration  either  with  or 
without  change  of  colour  of  the  affected  région  of  the  cord.  But 
microscopic  examination  of  sections  made  after  hardening  always 
detects,  in  the  compressed  part,  the  existence  of  transverse  inter* 


78  ALTEEATION  OP  THE  COED. 

stitial  myelitis,  recalling  tlie  characters  of  sclerosis  and  accompanied 
by  a  more  or  less  complète  destruction  of  the  nerve-tubes. 

The  plate,  whicli  I  place  before  you,  enables  you  to  recognise  the 
altérations  which  tlie  cord  présents  at  the  point  of  compression,  in 
the  case  of  a  woman  who  suffered  from  Pott's  disease  :  this  patient 
succumbed  to  an  intercurrent  disease,  at  a  time  when  the  consécu- 
tive paraplegia,  of  tvro  years'  standing,  was  most  marked  (see  Plates 
I  and  II). 

The  neuroglia  appears  transformed  into  a  dense  and  resisting 
connective  tissue.  The  trabeculae  which  it  forms  are  usually  thick- 
ened.  Generally,  the  meduUary  cylinders  of  the  nerve-tubes  hâve 
disappearedj  and  we  find  instead  groups  of  fatty  granulations  agglo- 
merated  under  the  form  of  granular  bodies.  Many  of  the  axis- 
cylinders  hâve  persisted  ;  some  of  them  even  seem  to  hâve  augmented 
in  size. 

In  short,  we  find  hère  again  the  characters  which  are  met  with  in 
certain  cases  of  primary  subacute  or  chronic  transverse  myelitis. 

But,  the  spinal  lésions  in  slow  compression  do  not  remain  con- 
fined  to  the  point  compressed  ;  they  extend,  following  the  laws  well 
known  since  the  labours  of  Tiirck,  above  and  below  this  point, 
along  certain  fascicles  of  the  cord  ;  above,  they  afiFect  the  posterior 
columns,  and  below,  the  latéral  columns.  Finally,  the  lésion 
influences  only  one  latéral  half  of  the  cord,  if  the  compression  itself 
be  hemilateral. 

Are  thèse  secondary  lésions  purely  passive  and  analogous  to 
that  which  the  section  of  a  nerve  détermines  ?  I  am  uuable  to  say. 
It  is  constant  that  they  show  themselves,  as  I  hâve  pointed  out,  in 
cases  of  primary  myelitis,  that  is  to  say,  in  cases  where  compression 
cannot  well  be  accused.  It  is  constant  also,  and  this  is  an  impor- 
tant peculiarity,  that  at  a  given  moment  the  lésions  in  question 
show  themselves  in  the  invaded  columns  with  ail  the  characters  of 
interstitial  sclerosis.  Hence,  we  hâve  not  to  deal  with  an  ascending 
and  descending  degeneration  only,  in  the  strict  acceptation  of  the 
Word,  but  with  a  true  sclerosis  or  consécutive  fasciculated  sclerous 
myelitis. 

II. 

Transverse  myelitis  with  consécutive  ascending  and  descending 
sclerosis, — snch,  in  short,  gentlemen,  are  the  necessary  efiects,  so 
to  speak,  of  slow  compression  of  the  cord,  whether  it  be  due  to 


CURABILITY  OF  POTT's  DISEASE.  79 

Pott^s  disease,  cancer,  tumours  of  ail  kinds,  or  yet,  again,  to  intra- 
spinal  tumours.  Hère  a  question  suggests  itself  :  Is  thé  marked 
disorganisation,  referred  to  above,  beyond  tlie  resources  of  nature 
and  of  art?  Can  a  cord  so  gravely  altered  never  résume  the 
whole  or  part  of  its  functions,  whilst  recovering  at  the  same  time, 
it  is  understood,  tlie  whole  or  portion  of  its  normal  texture,  tlie 
cause  of  compression  liaving  ceased  ? 

It  is  not  questionable  but  that  this  may  take  place,  as  regards 
Pott's  disease,  and  most  probably  the  same  would  hold  good  with 
respect  to  cases  of  tumours  were  it  not  the  nature  of  thèse  not  to 
rétrograde. 

The  curable  character  of  Pott's  disease,  which  has  been  well 
established,  particularly  by  MM.  Bouvier  and  Leudet,^  even  where 
it  entails  paraplegia,  allowed  us  already  to  foresee  that  this  might 
be  the  case. 

Some  classic  authors,  indeed,  seem  to  believe  that,  when  once  it 
showed  itself,  paraplegia  from  Pott^s  disease  scarcely  ever  retro- 
graded;  they  make  mention  only  of  cases  in  which  paralysis  of  the 
superior  extremities,  after  having  been  more  or  less  well  marked, 
improved  or  even  completely  disappeared  in  proportion  as  an 
abscess  by  congestion  was  developed  on  a  part  of  the  body. 

Thèse  assertions,  gentlemen,  would  give  you  a  very  false  idea  of 
the  future  of  paraplegia  from  Pott's  disease.  It  is  notorious,  in 
this  hospital,  that  paraplegia  from  Pott's  disease  is  often  cured,  is 
perhaps  mostly  cured,  in  the  conditions  in  which  we  see  it, 
even  when  symptoms,  which  allow  no  doubt  of  the  existence  of  an 
inveterate  myelitis,  hâve  been  exhibited  in  a  most  évident  manner, 
and  are  of  old  standing. 

I  cannot  say  if  the  Pott's  disease,  which  is  cured  in  such  cases, 
belongs  more  specially  to  one  or  other  of  the  forms  described  by 
M.  Broca.  Ail  that  I  can  afifirm  is  that  the  patients  are  persons 
who  hâve  surmounted  the  first  phases  of  the  disease,  and  whose 
gênerai  health  is  satisfactory.  And  I  can  also  assure  you  that  none 
of  those  patients  had  any  abscess  externally  discernible. 

Putting  this  aside,  the  paralysis,  I  repeat,  may  hâve  shown 
itself  as  complète  as  possible,  accompanied  by  insensibility,  by 
permanent  contracture,  and  may  hâve  persisted  without  change  for 
the  better  during  months  or  even  years. 

'  Leudet,  '  Curabilité  des  accidents  paralytiques  consécutifs  au  mal  ver- 
tébral de  Pott,  Soc.  de  Biologie,  1862-3,  t.  iv,  p.  loi. 


80  CONDITION  OF  COEÎ). 

I  can  introduce  to  you  two  patients  in  whom  this  happy  resuit 
has  been  obtained.  One  of  tliem  had  the  lower  extremities  com- 
pletely  paralysed  for  eighteen  months,  tlie  other  for  nearly  two 
years.  Both  of  them,  naturally,  hâve  retained  their  spinal  defor- 
mity  ;  but  both  hâve  recovered  the  complète  use  of  their  lower 
extremities  ;  for  the  last  two  or  three  years  they  hâve  been  able  to 
walk  without  feeling  of  fatigue,  and  can  go  long  distances  on  foot. 
In  other  words,  they  do  not  retain  the  slightest  remnant  of  their 
paraplegia.  In  this  institution  and  elsewhere,  I  hâve  already  met 
with  five  or  six  similar  cases.  In  such  instances^  the  cure  seems  to 
me  to  be  due  to  the  intervention  of  médical  art  ;  it  is  after  the 
application  of  the  actual  cautery,  in  punctuations  upon  the  hump, 
on  either  side  of  the  spinous  apophyses  that  the  cure  takes  place. 
I  do  not  believe  that  this  can  be  always  attributed  to  a  mère 
coincidence  ;  it  is  to  some  degree  a  foreseen  and  predicable  consé- 
quence. 

Well,  but  what  was  the  state  of  the  cord  on  a  level  with  the  com- 
pressed  point,  in  thèse  patients,  or  rather  what  is  its  condition 
still  ?  I  believe  I  shall  be  able  to  give  you  satisfactory  information 
with  respect  to  this  question.  The  changes  which  we  hâve  observed 
in  the  case  of  a  patient  named  Dup — ,  who  recently  succumbed  to 
coxalgia,  when  her  paraplegia  had  been  cured  two  years,  will  serve 
for  the  démonstration. 

In  this  woman's  case,  the  cord  at  the  part  where  compression 
had  taken  place  in  conséquence  of  Pott's  disease,  was  no  bigger 
than  the  barrel  of  a  goose-quill,  and  in  section  corresponded  to 
about  the  third  of  the  section  of  a  normal  cord,  examined  in  the 
same  région.  It  was  very  firm  in  consistence,  and  grey  in  colour; 
in  one  word,  the  cord  presented  ail  the  appearances  of  most 
advanced  sclerosis  (PI.  III,  fig.  i). 

Above  and  below  this  contracted  part,  the  white  fascicles,  in 
the  usual  direction  of  secondary  degenerations,  were  occupied  by 
grey  tracts. 

Between  thèse  appearances  which  the  contracted  part  of  the  cord 
présents,  when  examined  only  by  the  naked  eye,  and  the  phenomena 
observed  during  life,  there  seems  to  exist  a  most  striking  and 
singular  contradiction. 

The  recovery  of  function,  we  bave  said,  was  perfect  at  the  time  of 
death  ;  nevertheless,  at  that  period,  the  cord,  if  we  consider  the 
information  supplied  by  macroscopic  examination  only,  was  the  seat 


EE GENERATION  OF  NEEVE-TUBES.  81 

of  lésions  so  profound  that  it  seemed  literallj  interrupted,  in  one 
part  of  its  course,  by  an  apparently  scierons  band  in  which  it 
might  fairly  be  supposed  that  ail  trace  of  nerve-tubes  had  dis- 
appeared. 

Histology  shows  us  that  the  contradiction  is  not  a  real  one.  The 
connective  substitution  is  hère  only  in  appearance.  In  the  heart 
of  the  fibrous  tracts,  which  are,  indeed,  very  dense  and  very  thick, 
and  which  give  to  the  cord  its  grey  colour  and  hard  consistence, 
the  microscope  enables  us  to  discover  a  tolerably  large  quantity  of 
nerve-tubes,  furnished  with  their  axis-cylinders  and  their  medullary 
sheaths,  and,  consequently,  very  regularly  and  normally  constituted. 

By  means  of  thèse  nerve-tubes  it  was  that  the  normal  trans- 
mission of  the  commands  of  the  will  and  sensory  impressions  was 
carried  ont. 

Hère,  however,  we  meet  with  more  than  one  serions  difficulty. 

In  the  first  place,  how  bas  the  réparation  taken  place  of  those 
nerve-tubes  which  re-established  the  nervous  communications 
between  the  upper  and  the  lower  segment  of  the  spinal  cord? 
Did  a  complète  reproduction  of  the  nerve-filaments  happen,  or  was 
there  only  a  reappearance  of  the  medullary  sheaths  to  clothe  the 
denuded  axis-cylinders  ? 

Again,  as  I  pointed  ont  to  you,  the  section- surface  of  the  pièce 
ef  contracted  cord  scarcely  represented,  in  its  diameter,  a  third  of 
the  surface  of  a  normal  cord,  considered  in  the  same  région.  The 
uumber  of  nerve-tubes  was,  consequently,  much  below  the  normal 
standard  in  the  compressed  part  of  the  cord.  I  should  add  that 
the  grey  substance  was  no  longer  represented  in  this  part,  save  by 
one  of  the  horns  of  grey  matter  in  which  we  could  find  but  a  small 
number  of  nerve-cells  intact.  Nevertheless,  thèse  conditions,  so 
unfavorable  in  appearance,  had  sufficed,  I  repeat,  for  the  complète  re- 
establishment of  sensation  and  movement  in  the  inferior  extremities. 

Thèse  are  so  many  problems  of  pathological  physiology  which  I 
am  not  in  a  position  to  solve  at  présent,  and  so  I  confine  myself  to 
offering  them  for  your  méditation. 

III. 

We  are  now  in  a  condition  to  study,  with  advantage,  the  sym- 
ptoms  which  resuit  from  slow  compression  of  the  spinal  cord.  But, 
on  the  very  threshold  of  the  question,  the  necessity  of  establishing 
an  important  distinction  appears  before  us.     The  symptoms  which 

VOL.  II.  6 


83  STMPTOMS. 

are  directly  connected  witli  the  effects  of  the  interruption  of  the 
course  of  nerve-fibres  in  the  cord  are  almost  never  seen  completely 
isolated,  in  practice.  Almost  always  phenomena  are  superadded, 
whose  character  varies  according  to  the  nature  of  the  organic  lésion 
which  is  at  work.  And,  gentlemen,  a  thorough  knowledge  of 
thèse  phenomena  is  of  the  highest  interest  for  the  clinical  observer, 
for  this  it  is  which,  in  the  immense  majority  of  cases,  supplies  the 
éléments  of  the  diagnosis.  In  point  of  fact,  as  I  explained  to  you 
at  our  last  meeting,  the  conséquences  proper  to  spinal  compression 
itself  are  always  the  same,  whatever  be  the  cause  of  the  pressure. 
They  vary  but  little,  in  short,  save  in  proportion  to  the  degree  of 
the  compression,  or  according  as  it  affects  one  or  other  région  of  the 
cord.  Hence,  it  is  not  in  this  direction,  you  perceive,  that  we 
could  hope  to  discover  distinguishing  characters. 

IV. 

For  greater  clearness,  we  will  designate,  by  the  name  of  extrinsic 
symptoms,  those  phenomena  which  intermingle  with  the  peculiar 
symptoms  of  spinal  compression.  For  the  latter  we  specially  reserve 
the  name  of  intrinsic  symptoms.  Let  us  now  consider  the  first 
mentioned. 

In  order  to  facilitate  the  knowledge  of  thèse  extrinsic  symptoms , 
and  the  better  to  understand  the  cause  of  their  existence_,  let  us 
refer  to  the  topographie  study  of  the  région  in  which  ail  the  phe- 
nomena, that  we  are  about  to  describe,  are  produced. 

Proceeding  from  without  inwards,  after  the  extra-rachidian  soft 
parts  abundantly  provided  with  nerves,  we  encounter  the  différent 
parts  of  the  vertébral  column  in  which  numerous  nerve-filaments 
are  distributed,  and  which,  in  the  pathological  condition,  may 
become  the  seat  of  acute  pain.  Then  comes  the  adipo-cellular  layer 
{périméninge) ,  which  the  multiplied  nerve-filaments  that  accompany 
the  vertébral  sinuses  {7'ami  sinus  vertébrales,  Luschka)  peuetrate 
by  the  intervertébral  notches. 

Deeper  still,  we  find  the  envelopes  of  the  cord.  The  dura  mater 
first  présents  itself;  Purkinje,  Kôlliker,  Luschka,  hold  that  it  is 
devoid  of  nerve  branches  ;  Rudinger,  on  the  other  hand,  main- 
tains  that  it  possesses  them.  However  this  may  be,  according 
to  Haller  and  Longet,  the  dura  mater  is  insensible  in  the 
normal  state  j  but,  on  the  other  hand,  it  is  certain,  according  to 
Plourens,  that  in  the  pathological  condition,  that  is  to  say,  when 


SYMPTOMS.  83 

it  is  inflamed,  the  dura  mater  may  become  the  seat  of  acute  pain.;  as 
to  the  aracJmoid  membrane,  it  has  no  proper  nerves.  On  the  other 
hand,  ihs,  pia  mater  possesses  a  large  number  of  them. 

This  is  not  ail  y  et.  The  cord  appears,  to  some  extent,  endowed 
with  sensibility  in  its  posterior  columns,  at  ail  events,  if  we  are  ta 
judge  by  expérimental  conditions.  You  will  understand  from  this 
sumraary  sketch,  gentlemen,  that  the  différent  parts  we  hâve  just 
enumerated  may,  ail  of  them,  betray  their  suffering  by  more  or  less 
acute  pain.  However,  I  bave  purposely  neglected  until  now  the 
most  important  point. 

Prom  the  spinal  cord  arise  the  anterïor  and  posterior  roots  which 
traverse  the  pia  mater,  the  arachnoid,  and  finally  the  dura  mater, 
and  then  unité  to  form  the  original  trunks  of  the  mixed  nerves 
which  travel  some  distance  through  the  intervertébral  channels 
before  proceeding  to  spread  out  beyond.  Now,  ail  thèse  parts  are 
eminently  sensitive,  the  anterior  roots  excepted,  and  it  is  precisely 
because  of  the  irritation  which  they  undergo  on  account  of  the 
compression,  that  the  most  interesting  of  the  extrinsic  symptoms 
offered  to  our  study  are  developed. 

V. 

Itistrue  we  should  not  neglect  the  noteworthy  indications  which 
may  be  supplied  by,  i°,  the  présence  of  an  extra-rachidian  tumour 
(aneurism,  hydatid  tumour)  ;  2°,  the  discovery  of  a  déformation 
of  the  spinal  column,  presenting  itself  with  varied  characters  accord- 
ing,  for  instance,  as  it  is  connected  with  Pott's  disease  or  with 
vertébral  cancer;  3°,  nor  are  we  to  neglect  the  existence  of  local 
pain  corresponding  to  the  place  which  the  lésion  occupies,  and 
depending  on  irritation  of  the  bone  or  on  that  of  the  méninges. 
But,  above  ail,  we  must  closely  attend  to  the  symptoms  resulting 
from  irritation  of  the  roots,  or  of  the  peripheral  nerves.  Por,  it 
is  their  présence  which,  above  ail,  impresses  a  peculiar  physiognomy 
on  the  différent  forms  of  spinal  compression.  They  are  absent,  in 
fact,  as  a  rule,  in  those  cases  in  which  tumours,  oryet  other  lésions, 
become  primarily  developed  in  the  substance  of  the  spinal  cord. 
Hence,  M.  Cruveilhier  could  say,  with  justice,  that  acute  pain  is  a 
symptom  of  extra-spinal  lésions,  and  that  it  is  absent  in  crises  of 
intra-spinal  lésions.  Although  hère,  as  elsewhere,  exceptions  are  not 
rare,  the  rule  remains.  Dr.  Gull  is  of  the  same  opinion,  since  he, 
also,  asserts  that  this  is  a  characteristic  sign. 


84  PSEUDO-NEUEALGIAS. 

jàccording  to  tlie  foregoing,  gentlemen,  thèse  symptoms  always 
précède  (a  point  wortli  noting  as  it  lias  its  own  importance)  the 
appearance  of  tlie  intrinsic  symptoms  ;  so  that  often,  and  for  a  very 
long  period,  tliey  constitute  of  themselves  the  whole  disease,  or 
rather  ail  the  external  appearances  of  the  disease.  This  is  a  cir- 
cumstance  which  may,  in  practice,  be  the  occasion  of  a  multitude  of 
mistakes  which  require  to  be  carefuUy  shunned.  In  support  of 
this  assertion,  it  will  be  enough  for  me  to  remind  you,  for  instance, 
of  the  difficulties  in  diagnosing  vertébral  caries,  at  the  outset. 

The  symptoms  due  to  irritation  of  the  nerve  roots  or  of  the  peri- 
pheral  nerves  are  constant  or  little  short  of  it,  and  you  will  easily 
comprehend  it,  if  you  would  just  remark  that,  at  a  giveu  moment, 
an  extra-spinal  tumour,  whatever  be  its  starting  point,  cannot  fail 
to  encounter  the  nerve-roots,  or  the  mixed  nerves  in  tlieir  intra- 
rachidian  course,  and  to  cause  their  compression,  that  is  to  say, 
their  irritation,  at  least  in  the  early  stages. 

With  respect  to  the  extra-rachidian  tumours  which  tend  to 
approach  the  cord,  they  bring  about  an  analogous  resuit,  by  pro- 
ducing  irritation  of  the  nerve-trunks  after  their  exit  from  the 
rachis  (aortic  aneurism,  hydatids). 

VI. 

The  symptoms  in  question  are  commonly  designated  nnder  the 
name  oî  jjseitdo-neioralgic  ;  but,  in  reality,  almost  always,  at  least  at 
a  certain  period,  we  bave  hère  a  true  neuritis,  comparable  in  ail 
respects  to  that  which  arises  and  progresses  under  the  influence  of 
a  traumatic  lésion.  The  character  of  the  pain  is  the  samc  (burning 
pains).  The  absence  of  painful  points,  increased  by  pressure, 
which  is  one  of  the  objective  characters  of  neuralgias,  is  likewise  to 
be  remarked.  Pinally  comes  the  manifestation  of  the  séries  of 
trophic  disorders  which  scarcely  belong  to  neuralgias  properly  so 
called  ;  such,  for  instance,  are  zona,  pemphigoid  bullse,  and  even 
eschars,  in  the  cutaneous  région  ;  and  more  or  less  rapid  atrophy, 
paralysie,  and  contracture,  in  the  muscular.  Moreover,  the  inflam- 
niatory  nature  of  the  nerve-lesions  bas  been  several  times  clearly 
identified  by  M.  Bouvier,  araongst  others,  in  Pott's  disease,  and  by 
uiyself  in  vertébral  cancer. 

VII. 

But  let  us  leave  tliis  gênerai  point  of  view,  gentlemen,  to  descend 


INTRA-RAGHIDIAN  TUMOURS.  85 

into  the  concrète^  and  show  tlie  clinical  interest  whicli  beloiigs  to 
the  study  of  thèse  symptoms  ;  we  will  examine  them,  successively, 
in  the  three  principal  groups  which  follow  :  i°,  intra-rachidian 
tumours  ;  2°,  Pott's  disease  ;  3°^  vertébral  cancer.  It  will  be  easy 
afterwards  to  apply,  in  a  certain  measure,  to  the  other  forms  the 
results  which  this  first  study  is  about  to  furnish  us. 

The  principle,  in  fact,  is  always  the  same,  whatever  be  the  start- 
ing  point  of  the  pain  ;  the  latter  radiâtes  according  to  the  direction 
of  the  nerves  whose  roots  are  affected,  irritated,  compressed  ;  and  it 
conforms  generally  to  the  law  of  penplieral  sensation.  On  pres- 
sure, we  observe  some  varieties;  sometimes  the  pain  is  circum- 
scribed  within  a  more  or  less  limited  région  ;  sometimes,  on  the 
contrary,  the  course  of  the  nerve  appears  affected  throughout  its 
whole  extent. 

A.  lutm-rachidian  tumours. — The  pseiiclo-neur algie  pain  hère 
précèdes,  as  the  rule  is,  the  development  of  the  myehtic  symptoms 
properly  so-called.  The  nerves  adjacent  to  the  tumour  are  the 
first  compressed  ;  or,  rather,  the  cord  may  be  compressed  for  a 
certain  time  before  being  irritated,  and  before  manifesting  its  suf- 
fering  by  symptoms  proper  to  itself,  whilst,  as  to  the  nerves,  they 
seem  to  respond  almost  immediately  to  the  irritating  cause.  In 
such  a  case,  the  pain  often  occupies  but  a  very  limited  région  ;  it  is 
a  point  or  a  line  which  is  paiiiful,  uot  a  surface.  The  domain 
which  the  pain  affects  is,  other  things  being  eqaal,  limited  in  pro- 
portion as  the  turaoUr  is  small. 

The  pain,  it  is  well  understood,  is  on  the  right  side,  if  the 
tumour  be  on  the  right,  and  on  the  left,  if  the  tumour  be  on  the 
left  ;  it  is  bilatéral  (not  the  most  common  case  perhaps),  when  the 
morbid  growth  presses  equally  on  the  nerve  roots  of  both  sides  of 
the  cord. 

In  support  of  thèse  assertions,  gentlemen,  I  think  it  well  to 
quote  brielly  some  examples. 

1°.  In  the  case  of  a  patient,  observed  in  this  hospital,  named 
Gill — 5  we  had  to  deal  with  a  sarcoma  of  the  perimeningitic  layer 
which  penetrated  into  an  intervertébral  notch  of  the  left  side, 
and  extended  to  the  corresponding  pleura  ;  there  had  existed,  in 
this  patient,  a  fixed  painful  point  ou  the  left  of  the  thorax,  several 
months  before  the  formications  of  the  inferior  extremities,  which 
inaugurated  the  paraplegia,  had  sliown  themselves. 

2°.  I   borrovv    the   foUowing   example    from   Mr.    Ceyley,    an 


86  PSEUDO-NEUEALGIAS  IN  POïT  S  DISEASE. 

Englisli  author.^  Tlie  tumour,  a  psammoma,  was  situated  at  tlie 
eleventh  dorsal  vertebra,  and  compressed  the  cord.  The  patient 
had  constautly  complained  of  a  lixed  painful  points  towards  tlie 
left  iliac  fossa,  during  the  six  months  whicli  preceded  the  iirst 
appearance  of  formications  in  the  lower  extremities.  The  pecuhar 
seat  of  the  painful  point  in  this  patient  is  explained  by  the  fact  that 
the  last  intercostal  nerve,  whicli  the  tumour  pressed  upon,  gives  off 
terminal  branches  to  the  vicinity  of  the  iliac  crest. 

3°.  In  a  case  reported  by  Dr.  Bartels,^  an  intra-rachidian  hydatid 
tumour  compressed  the  left  half  of  the  lower  part  of  the  cervical 
enlargement.  During  three  months,  the  only  symptoms  observed 
were  pains  radiating  into  the  arm,  hand_,  and  shoulder,  of  the  left 
side,  and  accompanied  by  a  feeling  of  constriction  at  the  lower  part 
of  the  neck.  Not  till  the  erid  of  this  period  did  formication  super- 
vene  in  the  left  foot,  and  soon  after  came  the  other  symptoms  of 
spinal  compression. 

You  will  understand  that  such  or  such  other  nerve,  the  sciatic 
for  instance,  may  be  affected  in  the  same  manner  ;  that  dépends  on 
the  place  occupied  by  the  tumour.  The  seat  of  the  radiated  pain 
matters  little,  however.  What  is  important  is  the  fact  that  the 
symptom  in  question,  when  well  and  duly  identified  amongst  the 
prodromes,  will  be  sufficient  to  differentiate  paraplegia,  arising 
from  slow  compression,  from  a  primary  spinal  affection. 

B.  Pseudo'neîiralgkis  m  Pott's  disease. — We  will  only  touch 
lightly  on  the  description  of  pseudo-neuralgias  in  Pott's  disease, 
not  that  they  do  not  deserve  our  attention,  but  that  in  order  to 
treat  such  a  question  thoroughly  we  should  enter  into  numerous 
détails  which  it  would  be  indispensable  to  notice.  There,  in  fact,  lies  a 
large  portion  of  the  history  of  latent  vertébral  caries,  and  you  are 
not  ignorant  of  what  difficulties  we  meet  with  in  diagnosis,  during 
the  first  period  of  the  disease. 

The  organic  condition  has  not  been  completely  elucidated  hère. 
Probably  it  is  variable  ;  sometimes  the  nerve  lésion  is  at  the  dura 
mater;  sometimes  it  is  within  the  intervertébral  notch.  The  latter 
case  is  the  rarer,  if  it  be  exact,  as  several  authors  maintain,  that 
thèse  notches,  in  Pott's  disease,  never  give  way  so  much  as  to  allow 
of  the  compression  of  the  nerves  which  pass  tlirough  them. 

^  Ceyley,  '  Pathological  Society,'  t.  xvii,  p.  25,  1868. 

■  Bartels,  "Ein  Fall  von  Ecbinococcus  innerhalb  des  sackes  der  Dura  Mater 
•spinalis"  ('Deutsches  Archiv  fiir  klinische  Mediciii,'  vol.  v,  p.  180,  1869). 


OANOEEOUS  VERTEBRAL  DISEASE.  87 

Let  it  suffice  for  me  to  say,  gentlemen,  that  according  to  the 
seat  of  the  vertébral  disease,  the  gir die  pain,  or  the  appearance  of  a 
bracJdal  or  a  sciatic  neuralgia  frequently  précèdes,  by  a  long 
interval,  the  first  manifestation  of  the  spinal  symptoms,  properly 
so  called. 

The  disorders  resulting  from  the  pseudo-neuralgias,  in  patients 
affected  by  vertébral  caries,  may  extend  to  the  production  of 
cutaneous  éruptions,  such  as  zona,  as  was  observed  in  a  case 
reported  by  Dr.  Wagner,^  and  in  another  described  by  M. 
Michaud."  Muscular  atrophy  also  may  be  produced  ;  and  if  so,  it 
may  show  itself,  according  to  Dr.  Benedikt,^  without  paralysis,  pro- 
perly so-called  ;  electric  contractility  may  be  preserved,  whilst  elec- 
tric  sensibility  has  increased,  a  peculiarity  which  seems  to  prove 
that  muscular  atrophy  is  hère  really  the  resuit  of  an  irritative 
action. 

c.  But,  it  is  chiefly  on  the  study  oî pseudo-neuralgias  connected 
with  cancerous  vertébral  disease  that  I  should  désire  to  concentrate 
your  entire  attention.  Mauy  motives  lead  to  tins  décision.  In  the 
first  place,  we  hâve  hère  an  order  of  facts  which  is  yet  but  little 
known,  or  at  least  but  badly  known,  and  badly  interpreted,  not- 
withstanding  the  numerous  works  which  hâve  been  published  on 
the  subject.  In  the  second  place,  the  knowledge  of  thèse  facts  is, 
as  I  hope  to  demonstrate  to  you,  of  very  great  importance,  in  prac- 
tice.  Einally,  need  we  say  it  is  a  question  of  much  local  interest. 
It  was,  in  fact,  in  this  hospital  that  the  first  serions  studies  were 
undertaken  in  i^rance,  upon  the  subject  ;  the  first  foundations  were 
laid  by  my  master  and  friend,  M.  CazaHs.  It  was  he,  I  repeat  (and 
with  ail  the  more  willingness,  because  it  seems  to  hâve  been  for- 
gotten  in  a  récent  article),  who  demonstrated  the  anatomical  and 
physiological  reason  of  this  particular  species  of  pseudo-neuralgia, 
by  establishing  the  fact  that  it  results  from  the  pressure  which  the 
nerve-trunks  undergo  in  the  intervertébral  notches  ;  and  that  it 
does  not  dépend,  as  many  persons  still  seem  to  think,  on  compres- 
sion exercised  on  the  spinal  cord.  The  works  of  M.  L.  Tripier,* 
those  of  M.  Lépine,^  hâve  completed,  by  the  addition  of  some 

'  E.  Wagner,  '  Archiv  der  Heilkuude,'  4  heft,  1870,  p.  331. 

^  Loc.  cit. 

2  '  Electrotherapie,'  t.  ii,  p.  316. 

■*  Tripier  (L.),  'Du  cancer  de  la  colonne  vertébrale,'  &c.,  1867. 

*  Lépine  (R.),  'Bulletin  de  la  Société  Anat.,'  1867. 


88  PAINFUL  PARAPLEGIA. 

important  features,  my  first  observations  whicli  date  from  1865.^ 
But,  vue  hâve  been  preceded  abroad^  by  Mr.  Hawkins/  and  by  Dr. 
Leyden,^  with  whose  remarkable  vi'orks  I  was  not  acquainted  when 
my  memoir  was  published.  I  sliall  merely  mention  that  thèse 
authors  do  not  speak,  in  any  way,  either  of  the  mechanism  of  the 
lésion,  or  of  the  mode  of  production  of  the  symptoms  which  it 
détermines. 

Hère,  more  than  elsewhere,  the  distinction  between  the  pseudo- 
neuralgic  phenomena  and  the  symptoms  of  slow  spinal  compression 
is  of  the  highest  value.  Besides,  there  are  grounds  for  distinguish- 
ing  a  ])ecidiar  species  amongst  pseudo-neuralgias,  which,  in  cases 
where  it  exists,  give  a  spécial  physiognomy  to  the  disease.  With 
regard  to  this  form,  and  to  this  form  only,  it  is  just  to  say  with 
Hawkins,  Gull,  and  Leyden,  that  the  pains  in  vertébral  cancer  are 
almost  characteristic.  They  are  so  then,  in  reality,  up  to  a  certain 
point,  for  they  are  scarcely  found  with  the  same  character  except  in 
cases  of  extra-racliidian  tumotirs  tending  towards  the  cord,  as  in 
the  case  of  an  aneurism,  for  instance,  or  of  a  hydatid  tumour  which 
saps  away  the  vertébral  body  and  gets  into  contact  with  the  rachi- 
dian  nerves.  In  thèse  différent  cases,  the  organic  cause  is  always 
the  same  ;  it  is  the  compression,  the  acute  irritation  which  the 
nerve-trunks  undergo,  and,  in  the  case  of  vertébral  cancer  particu- 
larly,  the  nerve  lésions  produced  by  the  giving  way  of  the  softened 
vertebrse. 

Apart  from  this  circumstance,  vertébral  cancer  has  no  other  pains 
peculiar  to  itself  ;  or,  in  other  words,  the  cancer  may  penetrate  to 
the  cord  without  producing  other  pains  than  those  which  are 
developed  as  a  conséquence  of  ail  other  organic  lésions,  whatever 
they  may  be,  which  are  capable  of  deterraining  spinal  compression. 

To  sum  up,  there  are  :  1°,  latent  vertébral  cancers  ;  1°,  vertébral 
cancers  which  induce  compression  of  the  cord  almost  without  pre- 
dominating  pains  ;  3°,  lastly,  vertébral  cancer,  when  it  occasions 
the  softening  and  giving  way  of  the  vertebrse,  is  the  source  of  pains 

*  Charcot,  '  Sur  la  paraplégie  douloureuse  et  sur  la  thrombose  artérielle  qui 
surviennent  dans  certains  cas  de  cancer'  (Société  des  Hôpitaux,  Mars,  1865). 

2  C.  Hawkins,  "  Cases  of  Malignant  Disease  of  the  Spinal  Column,"  *  Med.- 
Chir.  Transactions,'  t.  xxiv,  p.  45,  1845. 

^  E.  Leyden,  "  Ueber  Wirbelkrebs,"  in  '  Annalen  der  Charité  Kranken- 
haus,'  i'^  Band,  3  heft,  p.  54;  consult  also  Black,  '  Centralblatt,'  1864,  p. 
493;  ïh.  Simon,  "  Paraplegia  dolorosa.  Aus  der  Allgemeiue  Krankenhaus  zu 
Hamburg,"  in  'Berlin.  Klin.  Wochens.,'  Nos.  35  and  36,  1870. 


PAINFUL  PARAPLEGIA.  89 

which  are  almost  spécifie.  This  giving  way  of  the  vertebrse  may 
exist  alone  or  be  accompanied  by  the  ordinary  symptoms  of  com- 
pression of  the  cord  ;  but,  I  do  not  hesitate  to  say,  as  regards  the 
case  in  point,  that  the  fact  of  compression  of  the  nerves  is  much 
more  interesting,  in  clinical  practice,  than  that  of  spinal  com- 
pression. 

The  assertions  which  I  hâve  just  made  to  you  are  founded  on 
repeated  observations,  some  of  which  hâve  been  pubhshed  in  the 
thesis  of  M.  L.  Tripier  :  in  thèse,  the  organic  lésion  in  question 
existed  with  the  whole  group  of  characteristic  symptoms,  without  the 
cord  being  at  ail  involved,  Leyden  and  Hawkins  mention  facts  of 
the  same  kind;  and,  since  the  appearance  of  M.  Tripières  thesis,  I 
hâve  several  times  verified  the  accuracy  of  my  pathogenic  interpré- 
tation. The  compressed  nerves  in  such  cases  are  red  and  swollen, 
but  yet  without  any  very  serions  histological  change  ;^  in  truth  our 
means  of  investigation  as  regards  this  point  are  still  comparatively 
very  coarse.  The  nerves  do  not  become  atrophied  and  undergo 
granular  fatty  degeneration  until  very  late  in  the  disease.  Never, 
so  far  as  my  expérience  goes,  did  I,  under  such  circumstances,  see 
the  cancerous  infiltration  of  the  nerve,  which  several  authors  hâve, 
I  believe,  rather  lightly  invoked. 

VIII. 

It  now  becomes  my  duty  to  describe  the  symptoms,  to  which  I 
hâve  desired  to  call  your  attention,  in  a  spécial  manner.  I  hâve 
proposed  to  group  thèse  symptoms  under  the  désignation  oipainful 
paraplegia  of  cancerous  patients?  This  dénomination  of  painful 
paraplegia  T  hâve  borrowed  from  M.  Cruveilhier,  who  clearly 
recognised  this  genus  of  symptoms,  without,  however,  divining  their 
interprétation.^  The  name  applies  with  exactness  only  in  cases 
where  the  lumbar  vertébral  région  is  affected  to  a  certain  extent. 
This,  indeed,  is  what  most  commouly  happens. 

But,  as  a  matter  of  fact,  several  otlier  combinations  are  possible. 
In  the  first  place,  the  vertebrae  having  given  way,  on  one  side  espe- 

^  Charcot  et  Cotard,  "  Sur  un  cas  de  zona  du  cou  avec  altération  des  nerfs 
du  plexus  cervical  et  des  ganglions  correspondants  des  racines  spinales  pos- 
térieures," '  Société  de  Biologie/  xvii,  1866,  p.  41. 

^  Charcot,  "Sur  la  paraplégie  douloureuse  qui  surviennent  dans  certains  cas 
de  cancer,"  '  Bulletin  de  la  Société  Médicale  des  Hôpitaux,'  loc.  cit. 

^  Cruveilhier,  'Atlas,'  32*-'  livr,,  p.  6. 


90  CHAEAOTER   OP    PAINS. 

cialljj  there  can,  in  conséquence,  be  only  a  painful  hemi-paraplegia  ; 
or  else,  again,  the  concomitant  pains  and  phenomena  may  occupy  the 
brachial  plexus  or  the  cervical  plexus,  when  the  lésion  bears  upon 
the  cervical  vertébral  région.  At  other  times,  finally,  the  pains 
show  themselves  exclusively  limited  to  the  distribution  of  this  or 
that  nerve-trunk.  It  is  also  important  to  remark  that,  whatever  be 
the  place  in  which  it  shows  itself,  how  circumscribed  soever  it  may 
be,  the  pain  in  cases  of  this  kind  présents  itself  absolutely  with  the 
same  characters  as  in  painful  paraplegia  properly  so  called. 

Let  us  suppose  that  the  lumbar  vertébrée  are  affected,  this  being, 
as  you  know,  the  most  common  occurrence,  and  that  thèse  bave  been 
invaded  throughout  by  the  cancer,  on  the  right  side  as  on  the  left, — 
conditions  which,  in  fact,  you  will  find  reproduced  in  one  of  the 
patients  whom  I  shall  introduce  to  you  in  a  moment; — now,  in 
such  a  case,  the  following  symptoms  are  to  be  noted  : 

Acute  ji^am,?  exist  ;  some  constricting  the  lower  part  of  the  abdo- 
men like  a  girdle,  others  spread  along  the  course  of  the  crural  and 
sciatic  nerves,  from  tlieir  spinal  origin  to  their  peripheral  extre- 
mities. 

There  is  h?/perœslhesia  of  the  integuments,  in  points  corres- 
ponding  to  the  distribution  of  the  painful  nerves.  This  hyperses- 
thesia,  generally,  is  so  great  that  the  least  touch  is  extremely 
painful. 

The  pains  in  question  are  permanent,  or  nearly  so,  but  they  become 
exasperated  in  paroxysms,  which  are  most  severe  at  night,  and  some- 
times  assume  a  periodic  character.  Movements  in  bed,  whether 
they  be  active  or  passive,  provoke  the  manifestation  of  thèse  pains 
or  exasperate  them.  The  same  holds  good,  a  fortiori,  as  regards 
standing  and  walking,  which  soon  become  wholly  impossible.  Hence 
there  results  a  kind  of  impotency,  which  does  not  arise  from  a 
decrease  of  muscular  strength,  for,  in  bed,  when  the  pains  do  not 
happen  to  be  too  acute,  the  movements  of  the  lower  extremities  are 
performed,  if  the  patient  be  not  very  much  weakened,  as  in  the 
normal  state. 

During  the  paroxysms,  the  pains  are  truly  frightful.  The  patients 
compare  them  to  those  which  would  be  caused  by  the  crushing  of  the 
bones,  the  bitiiig  of  the  deep-seated  parts  by  some  huge  animal,  etc. 
It  is  a  fact  worth  noticing  that  we  do  not  succeed  in  calmirig  them, 
except  with  great  difficulty,  by  the  use  of  high  doses  of  narcotic 
substances.      It  should  also  be  remarked  that,  without  appréciable 


ZONA,  ANiESTHESIA,  ETC.  91 

cause,  spontaneous  improvements  take  place,  as  regards  thèse  pains, 
the  physiological  reason  of  which  completely  escapes  us. 

To  thèse  phenomena  may  be  added,  especially  in  the  more 
advanced  stages  of  the  disease,  a  certain  number  of  disorders, 
amongst  vvhicli  I  would  specially  mention  the  éruptions  of  zona^ 
which  are  produced  along  the  course  of  particularly  painful  nerves, 
— a  cutaneous  ancesthesia,  circumscribed  in  patches  and  developing 
in  spite  of  the  persistence  of  pains  in  the  domain  of  the  affected 
nerves  {anœstliesla  dolorosa), — a  more  or  less  manifest  atrophj  of 
the  muscular  masses,  and  finally,  contracture  supervening  in  a  certain 
number  of  muscles. 

Lastly,  I  would  point  out  to  you  that  a  deformity  which  causes 
the  spine  to  describe  a  curve,  having  a  long  radius,  and  that  a  local 
vertébral  pain  which  pressure  or  percussion  provokes  or  exaspérâtes 
very  distinctly,  are  concomitant  symptoms  which  you  must  not 
neglect  to  seek  for  witli  care,  because  they  may  elucidate  the  situa- 
tion and  are,  besides,  frequently  to  be  found. 

Thèse  several  symptoms,  gentlemen,  may  be  for  many  months  the 
only  révélation  of  the  cancerous  vertébral  disease  ;  but  the  symptoms 
of  paralysis  by  com]^  itssic:  of  the  cord  may  become  superadded. 

However  this  may  be,  when  the  phenomena  of  painful  paraplegia 
appear  with  the  characters  just  described,  it  will  be  right  to  examine 
whether  there  do  not  exist,  in  some  part  of  the  organism,  some 
other  manifestation  of  the  cancerous  di  ithesis  ;  for,  as  you  are 
aware,  vertébral  cancer  is  usually  secondary,  deuteropathic.  Now, 
in  this  investigation,  we  may  find  ourselves  confronted  by  more  than 
one  difliculty  of  a  kind  calculated  to  set  the  diagnosis  astray.  I  will 
confine  myself,  at  présent,  to  calling  your  attention  to  the  following 
circumstance,  which  I  hâve  recently  witnessed.  It  may  happen 
that  the  patients  hâve,  in  the  breasts,  certain  atrophie  indolent 
cancers,  to  which  they  do  not  pay  the  least  attention.  I  was  con- 
sulted,  a  few  days  ago,  by  a  lady  who  liad  been  suffering  for  several 
months  with  a  cervico-brachial  neuralgia  ;  in  reality,  as  you  will 
see,  it  was  a  pseudo-neuralgia  ;  it  was  extraordinarily  painful,  and 
had  absolutely  resisted  ail  the  therapeutic  agencies  which  had  been 
tried.  Struck  by  the  spécial  character  which  the  pain  presented,  and 
remembering  the  facts  observed  in  La  Salpôtrière,  I  inquired  if  any 
mammary  lésion  existed.  I  was  answered  in  the  négative,  but  I 
thought  it  right  to  pursue  the  matter,  and  examine  how  things 
'  Cliarcot  et  Cotard,  loc.  cit. 


92  DIFFICULTIES  OF    DIAGNOSIS. 

stood  for  myself.  To  the  great  astonishment  of  the  patient^  I  dis- 
covered  that  one  of  the  breasts  was  deformedat  a  spot,  very  circum- 
scribed  indeed,  owing  to  a  rétraction  of  the  parts  consécutive  on 
the  puckering  occasioued  by  an  atrophie  schirrus.  Quite  recently, 
an  English  physician,  whose  narae  has  escaped  my  memory,  pub- 
lished  a  similar  case  in  a  volume  of  the  '  Proceedings  of  the  Patho- 
logical  Society  of  London/  Thèse  facts  will  suffice,  I  hope, 
gentlemen,  to  show  you  clearly  how  attentive  and  circumspect, 
under  such  circumsiances,  it  is  necessary  to  be  in  the  examination 
of  patients. 

On  the  other  hand,  you  must  not  go  so  far  as  to  beliere  that  the 
pains  of  cancerous  vertébral  disease,  even  when  they  appear  with 
ail  the  attributes  just  defined,  are  absolutely  spécifie  and  adapted 
to  guide  us  to  a  diagnosis  without  trouble.  Par  from  it  :  difficulties 
may  supervene,  but,  generally,  they  are  not  insurmountable. 
Amongst  the  affections  which  may  mislead,  on  account  of  the  pains 
that  accompany  them,  I  hâve  already  mentioned  the  aortic  aneurisms 
and  the  hjdaûd  hysts,  when  thèse  tumours  are  so  disposed  as  to 
compress  and  irritate  the  spinal  nerves.  I  «dll  now  refer  to  the 
existence  of  osteomalacia,  cervical  hypertropQic  pachymeningitis, 
and  lastly,  a  neurosis,  sjnnal  irritation. 

Senile  osteomalacia,  as  well  as  that  of  the  adult,  occasionally 
recalls,  by  the  character  of  the  accompanying  pains,  the  symptom- 
atology  of  the  vertébral  disease  of  cancerous  patients.  Hyper- 
trophie cervical  meningitis,  in  the  first  period,  is  also  often  accom- 
panied  by  somewhat  analogous  pains  ;  the  same  may  be  said  of  that 
strange  and  curions  affection,  which  is  sometimes  called  spinal 
irritation,  and  which  some  physicians  hâve  very  wrongly  sought  to 
banish  from  our  nosological  lists, — but  at  présent  I  désire  to  confine 
myself  to  calliiig  your  attention  to  thèse  diagnostic  difficulties.  I 
propose  at  another  opportunity  to  return  to  the  subject. 


LECTURE  VII. 

ON  SLOW  COMPRESSION  OF  THE  SPINAL  CORD.  SYMPTOMS. 
DISORDERS  OF  MOTILITY  AND  OF  SENSIBILITY  CONNECTED 
WITH  SPINAL  COxMPRESSION.  SPINAL  HEMIPLEGIA  AND 
HEMIPARAPLEGIA,  WITH  CROSSED  ANJLSTHESIA. 

SuMMARY. — Lésions  of  the  cord  at  tlie  point  qf  conijjressioni  They 
occiipy  the  ichole  extent  of  the  cord,  in  a  transverse  direction,  or 
onlij  affect  one  of  its  latéral  Jialves. 

First  case  :  Succession  of  symptoms.  Prédominance  of 
motor  disorders  at  the  outset  ;  paresis,paralysisioitJiJlaccidity, 
temporary  rigidity , permanent  contracture  qf  limbs,  augmentation 
of  reflex  excitabiUty.  Disorders  of  mictnrition  ;  Budge's 
theory.  Modification  qf  sensibilify  ;  delay  in  the  transmission 
of  sensations  ;  dysœsthesia.     Associated  sensations. 

Second  case  :  Lésions  hearing  on  one  of  the  latéral  halves  of 
the  spinal  cord.  Lésion  circumscrihed.  Spinal  hemiparaplegia 
loith  crossed  anuesthesia  ;  its  characters.     Spinal  hemiplegia. 

Gentlemen, — Before  pursuing  our  studies  on  slow  compression 
of  the  spinal  cord,  allow  me  to  offer  for  your  inspection  tlie  anato- 
mical  préparations  relating  to  this  subject,  which  we  owe  to  tlie 
kindness  of  Dr.  Liouvillc. 

In  paraplegia  from  Pott's  disease,  as  I  told  you,  the  déformation 
of  the  spiue  is  not,  as  a  gênerai  rule,  the  agent  of  the  compression 
which  the  cord  undergoes  ;  the  spinal  cord  may  even,  in  such  a 
casCj  be  compressed  without  the  vertébral  column  j)resenting  the 
slightest  trace  of  deformity.  This  préparation  of  Dr,  Liouville 
furnishes  plenary  évidence  of  the  fact.  The  spine,  as  you  perceive, 
was  not  at  ail  deformed  hère,  although  the  bodies  of  several  verté- 
brée were  deeply  altered.  The  anterior  vertébral  ligament,  on  a  level 
withthe  osseous  lésion,  was  as  if  dilacerated,  and  the  caseous  matter, 
according  to  the  mechanism  indicated  by  M.  Michaud,  had  corne 


94  ANATOMICAL   CONDITIONS. 

into  contact  with  the  dura  mater,  which,  in  conséquence,  presented 
on  corresponding  points  a  remarkable  thickening  {external  caseons 
pachymemngit'is).  It  was  evidently  tliis  thickening  of  the  dura 
mater  which  had  determined  the  spinal  compression.  The  latter 
was  clinically  represented  by  symptoms  of  paralysis  which,  as  is 
usual,  were  preceded,  many  months  before,  by  pseudo-neuralgic  pains 
simulatiag  intercostal  neuralgia.^ 

I  résume  now  tlie  course  of  our  conférences. 

The  question  is,  you  are  aware,  to  indicate  the  symptoms  which 
directly  arise  from  slow  compression  of  the  spinal  cord.  We  are 
about,  I  must  not  conceal  it  from  you^  to  enter  upon  a  path  long 
to  travel  and  bristling  with  difficulties  of  every  kind  ;  but  I  hope 
that,  by  planning  out  proper  stages,  we  may  arrive  at  our  goal  with- 
out  overmuch  fatigue. 


I. 

Let  us  recall  to  memory  the  anatomical  conditions  in  référence  to 
which  I  had  to  enter  into  some  détails.  The  cord,  you  hâve  not 
forgotten,  is  compressed,  strangled  as  it  were,  in  one  part  of  its 
course.  Now,  if  this,  in  the  early  period,  is  but  a  purely  me- 
chanical  phenomenon,  soon,  in  the  immense  majority  of  cases,  the 
éléments  which  compose  the  spinal  nerve-centre  react,  after  their 
manner.  On  a  level  with  the  compressed  part,  the  lésions  of  trans- 
verse wyelitis  from  compression  are  produced  ;  whilst,  above  and 
below  this  point,  develop,  according  to  the  laws  we  hâve  stated,  the 
altérations  of  secondary  fasciculated  sclerosis,  which  occupies,  in 
the  first  case,  the  médian  portion  of  the  posterior  columns,  and,  in 
the  second  case,  the  hindermost  part  of  the  latéral  columns. 

'  Thèse  anatomical  préparations  were  obtaincd  in  Professor  Béhier's  wards. 
The  patient  was  about  Of'ty  at  the  time  of  death.  Before  beinj;  seized  with 
paraplegia  hc  had,  for  several  months,  experienced  localised  ))aius  in  the 
course  of  the  thoracic  intercostal  nerves,  which  for  a  moment  had  induced  the 
belief  that  he  suffered  from  simple  intercostal  ncuralgia.  Later  on,  on  account 
of  the  persistence  and  character  of  the  pains,  the  opinion  was  expressed  that 
the  neuralgia  was  symptomatic,  though  it  was  found  impossible  as  yet  to 
décide  as  to  the  nature  of  the  primary  disease.  Then  supervencd  the  para- 
plegia, which  definitely  elucidated  the  diaguosis.  The  spine  preserved  its 
régulai-  form  until  the  fatal  end.  Again,  in  Professor  Béhier's  wards,  M. 
Liouville  bas  recently  observed  another  case  of  Pott's  disease,  likewise  without 
deformatioa  of  the  vertébral  column. 


EXTEINSIO    STMPTOMS.  SS- 

II. 

Let  us  consider  then  the  state  of  tbings  as  observed  under 
ordinary  conditions  ;  as  we  proceed,  we  will  note  exceptional  cir- 
cumstances,  and,  for  greater  clearness,  let  us  take  as  our  example 
the  commonest  case,  that  in  whîcli  the  lésion  occupies  anj  point  in 
the  dorsal  région  of  the  spinal  cord. 

It  is  time  now  to  make  a  first  distinction,  Sometimes  the 
compression  affects  the  whole  substance  of  the  cord  transverselj  ; 
sometimes,  on  the  contrary,  it  bears  upon  only  one  of  the  latéral 
halves  of  the  nerve  column.  It  is  the  first  case  which,  by 
the  way,  is  far  the  more  fréquent,  which  shall  now  engage  our 
attention. 

III. 

Let  us  thoroughly  reahse  the  situation  which  we  are  about  to 
examine.  For  several  weeks,  or  yet  longer  occasionally,  the  sym- 
ptoms  termed  extrinsic  j^ndamongst  them  the  pseudo-neuralgic  pains, 
alone  oceupy  the  scène.  In  the  hypothesis  which  we  bave  adopted, 
it  is  understood  that  we  bave  only  to  deal  with  organic  lésions 
primarily  situated  externally  to  the  cord, — this  nerve- centre,  then, 
bas  not  yet  exhibited  any  sufFering.  What  are  the  symptoms  which 
are  about  to  inaugurate  the  séries  of  new  phenomena  ?  Shall  they 
be  disorders  of  motion  or  dérangements  of  sensation  ?  The  order 
of  succession,  gentlemen,  is  difficult  to  détermine,  in  the  actual 
state  of  tbings,  for  lack  of  observations  collected  with  the  spécial 
intention  of  elucidating  this  particular  fact.  That,  however,  is  a 
matter  of  détail  somewhat  secondary  in  importance  as  regards 
practical  purposes,  but  which,  nevertheless,  is  not  without  some 
interest  when  theoretically  considered. 

In  point  of  fact,  formications,  pricklings,  sensations  of  beat  and 
cold  in  the  lower  extremities,  if  they  constituted  the  first  symptoms, 
would  necessarily  indicate,  according  to  the  theory,  that  the  con- 
ductors  of  feeling,  that  is  to  say  the  grey  substance,  had  undergone 
an  important  morbid  change  from  the  outset;  for,  it  bas  Ijeeu 
shown,  by  experiments,  that,  under  normal  conditions,  the  grey 
substance  does  not  provoke  any  kind  of  sensation  when  excited 
by  stimuli.  On  the  other  hand,  purely  motor  disorders,  paresis  or 
more  or  less  complète  paralysis  of  the  lower  extremities,  are  phen- 
omena which  raay  be  produced  apart  from  ail  change  in  the  properties 


96  MOTOE  DISOEDERS. 

of  the  éléments  of  the  cord,by  the  simple  fact  of  a  mère  mechanical 
interruption  of  the  continuity  of  the  nerve-fibres. 

However  this  may  be,  gentlemen,  the  reality  appears  to  be  that 
sometimes  the  motor  disorders  (paresis  of  the  lower  limbs),  some- 
times  the  sensory  disorders,  and  particularly  the  sensations  referred 
to  the  periphery,  and  indicating  irritation  of  the  grey  substance 
(prickling,  formication,  feeling  of  constriction,  articular  pains),  take 
the  lead  in  the  procession  of  symptoms, 

IV. 

Disorders  of  motion,  in  any  case,  do  not  deiay  to  predominate, 
in  the  first  stages  at  least,  over  the  sensory  dérangements.  Leav- 
ing  aside  the  few  subjective  phenomeua  just  mentioned,  the  transmis- 
sion of  sensory  impressions  is  long  carried  on  in  a  physiological 
manner,  when  the  power  of  motion  is  profoundly  modified,  and 
it  is  even  rare  for  such  transmission  to  be  ever  completely  inter- 
rupted,  or  indeed  very  gravely  involved. 

It  would  seem  that  the  grey  matter,  situated  in  the  centre  of  the 
cord,  is  protected  against  the  influence  of  causes  of  irritation 
coming  from  the  periphery.  This  is  a  pecuharity  which  has  long 
been  recognised  by  clinical  observation,  and  which  marks  a  contrast 
with  what  takes  place  in  cases  of  spontaueous  myelitis,  or  of  intra- 
spinal  tumours,  cases  in  which  thèse  lésions  usually  occupy  the 
central  portion  of  the  cord  from  the  commencement. 

Let  us  delay  a  moment  over  the  disorders  cf  moiilïiy. 

A.  In  the  first  degree,  %\m^t paresis  is  observed,  but  this  is  soon 
transformed  into  more  or  less  absolute  paralysis,  with  flaccidity  of 
the  limbs,  or,  in  other  terms,  without  muscular  rigidity. 

This  phenomenon  in  connection  with  the  interruption  of  the 
white  columns,  and  of  the  latéral  columns  especially,  is  in  confor- 
mity,  you  perceive,  with  the  results  of  expérimental  pathology. 

B.  At  the  end  of  some  days  or  of  some  weeks,  later  in  certain 
cases,  sooner  in  others,  there  supervene,  in  the  paralysed  members, 
jerks,  cramps,  a  temporary  rigidity  of  the  muscular  mass, — so  many 
symptoms  attaching  also  to  lésion  of  the  latéral  columns,  but  which 
already  indicate  that  a  source  of  excitation  has  taken  up  its  abode 
in  the  fascicles.  Thèse  are,  in  short,  the  first  symptoms  which 
can  be  referred  to  descending  myelitis  of  the  latéral  columns. 

c.  Lastly,  supervenes  permanent  contracture  of  the  limbs,  which 
almost  never  fails  to  exist  at  a  certain  period  of  the  diseasc,  and 


MOTOE   DISOEDEES.  97 

wliich  appears  to  be,  also,  coniiected  with  ihe  scierons  lésion, 
which  tho  latéral  columiis  présent  in  tlie  inferior  section  of  the 
cord.  It  is  tlie  rule  that  this  contracture  shall  first,  for  some  tirae, 
maintain  the  paralysed  limbs  in  a  posture  of  forced  extension,  but 
sooner  or  later,  generally  speaking,  a  posture  of  forced  flexion 
supervenes. 

D.  In  this  phase  of  the  disease,  under  the  combined  influence  of 
the  suppression  of  the  moderator  influence  of  the  brain,  and  prob- 
ably,  also,  because  of  the  irritation  of  which  the  grey  substance,  in 
its  turn,  becomes  the  seat,  the  reflex  properties  of  the  inferior  seg- 
ment of  the  cord  are  augmented.  Then,  we  see  the  paralysed 
members  rise  and  become  convulsed  at  the  least  touch,  or  when 
the  patient  micturates  or  goes  to  stool. 

I  will  not  dwell  upon  those  disorders  of  motility  which  to-day 
are  the  common  knowledge  of  ail.  I  will  confine  myself  to  makmg 
you  observe  that  the  intensity  of  permanent  contracture  of  the 
extremities,  and  especially  of  contracture  with  flexion,  is  generalîy 
more  marked  in  myelitis  froin  slow  compression  than  it  is  in  spon- 
taneous  myelitis. 

The  same  rnle  holds  good  with  respect  to  the  augmentation  of 
the  reflex  properties  of  the  cord.  Still,  it  will  not  do  to  make  of 
this  différence,  the  cause  of  which  completely  escapes  us,  an  abso- 
lute  diagnostic  charactcr. 

E.  It  is  the  rule,  also,  in  myelitis  from  compression,  at  ail 
events  when  the  dorsal  région  is  involved,^  that  the  bladder  shall 
préserve  to  a  grcat  extcnt  its  functional  integrity  duriug  a  compara- 
tively  lengthy  lapse  of  time;  but  more  or  less  marked  vesical 
dérangement  may  flnally  supervene.  With  respect  to  this  subject, 
a  distinction  must  bc  drawn. 

If  the  compression  is  seated  high  up,  towards  the  middle  of  the 
dorsal  région  for  instance,  we  generalîy  remark  a  difïiculty  in  the 
émission  of  urine.  This  difîiculty  seems  due  to  this,  that  the 
muscles  which  serve  as  sphincters  remain  in  a  state  of  permanent 
spasm.  The  will  does  not  readily  overcome  this  permanent  con- 
traction, and  the  involuntary  émission  of  urine  which  sometimes 
occurs,  in  such  cases,  happens  by  overflow  as  it  is  called. 

On  the  contrary,  if  the  lésion  is  situated  low  down,  towards  the 
upper   portion   of    the   lumbar   région,    it   may  happen    that   the 

^  Holmes,  '  A  System  of  Surgcry,'  t.  iii,  p.  858,  "  Inclusiou  of  the  Spinal 
Cord  in  Caries  of  the  Spinc." 

VOL.   II.  7 


98  THEORY  OF  BUDGE. 

sphincters  sliall  be  paralysed,  in  a  continuons  manner,  and  tlien  the 
urine  flows  involuntarily. 

It  is  possible,  in  some  measure,  to  account  for  this  différence, 
which  seems  singular  at  first  glance,  if  we  refer  to  the  theory, 
founded  on  expérimental  research,  which  Dr.  Budge  has  recently 
published  with  respect  to  the  mode  of  action  of  the  central  nervous 
System  on  the  functions  of  the  bladder.^ 

In  reality,  according  to  Dr.  Budge,  there  exists  no  other 
sphincter  of  the  bladder  than  the  urethral  muscles  (the  constrictor 
urethrse  and  bulbo-cavernosus).  The  nerves  which  cause  the 
bladder  to  contract  proceed  from  the  crura  cerebri.  Passing  by  the 
restiform  bodies,  they  may  be  experimentally  followed  in  the 
anterior  columns  of  the  cord  as  far  as  the  exit  of  the  3rd — 5th 
sacral  pairs.  The  nerves  which  cause  the  muscles  of  the  urethra  to 
contract,  follow,  still  according  to  îlerr  Budge,  nearly  the  same 
course,  and  they  offer  this  peculiarity,  that  they  are  modified  in  the 
normal  state  by  a  reflex  influence  which  is  communicated  to  them 
by  the  centripetal  nerves  coming  from  the  bladder.  Consequently, 
a  reflex  act  is  produced,  the  effect  of  which  is  to  détermine  the  per- 
manent contraction  of  the  urethral  muscles,  but  which  may  be 
modified  or  even  annulled  by  a  sort  of  inhibition  which  the  will 
commands. 

According  to  this  view,  every  lésion  which  should  hâve  the  efPect 
of  interrupting  in  the  cord  (down  to  the  exit  of  the  3rd,  4th,  and 
5tli  sacral  pairs)  the  course  of  the  nerves  that  proceed  either  to  the 
bladder,  or  to  the  urethra,  should  likewise  hâve  the  resuit  of  allow- 
ing  the  reflex  act  which  commands  the  constant  closure  of  the 
sphincter  to  subsist  ;  hence  it  is  that  lésions  of  the  cervical  and 
dorsal  cord  produce  the  permanent  spasm  of  the  vesical  sphincter 
which  is  observed,  in  certain  cases  of  spinal  compression. 

If,  on  the  other  hand,  the  lésion  is  seated  lower  down,  the  con- 
ditions of  the  reflex  act  in  question  no  longer  subsist;  the  sphincter 
is  paralysed  in  a  constant  manner,  and  the  urine  then  incessantly 
dribbles  ont,  drop  by  drop,  the  action  of  the  muscles  of  the  bladder 
no  longer  encountering  any  obstacle. 

I  am  not  unaware,  gentlemen,  that  Dr.  Budge's  theory  is  not  yet 
classic, — far  from  it,  the  expérimental  facts  on  which  it  is  based 

^  Budge,  '  Zeitschr.  f.  rat.  Heilk./  xxi,  p.  5  und  174;  "  Ueber  die  Reiz- 
barkeit  der  Vordereu  E,uclcenmarksstrànge"  ('  Pflûger's  Archiv  fur  Physiolo- 
gie,' Bd.  il,  p.  511). 


SENSORT   DISORDESS.  99 

require  to  be  themselves  verified.  Nevertheless,  I  thought  it  my 
duty  to  state  it  succinctly  because,  in  my  opinion^  it  explains  better 
than  any  other  theory,  at  présent  known,  the  facts  revealed  by 
clinical  observation. 


As  I  told  you,  a  little  ago,  sensation,  in  paralysis  from  compres- 
sion, does  not  generally  become  gravely  altercd  until  very  late  ; 
unless,  indeed,  the  lésion  be  one  whicli  primarily  occupied  the 
central  portion  of  the  cord.  However  this  may  be,  hère  is  a  sum- 
mary  of  some  peculiarities  relative  to  disturbances  in  the  trans- 
mission of  sensory  impressions  which  show  themselves  by  pré- 
férence, but  not  however  exclusively,  in  paralyses  from  spinal  com- 
pression ;  they  are  not  seen,  as  you  must  hâve  anticipated  after  what 
has  been  said,  except  in  cases  in  which  compression  reaches  a 
high  degree  of  intensity. 

In  the  iirst  place,  I  would  mention  the  delay  in  the  trans- 
mission  of  sensations,  a  curious  phenomenon  Which,  if  I  mistake 
not,  was  first  noticed  by  M.  Cruveilhier.^  Thirty  seconds  may 
elapse,  as  I  hâve  once  observed,  between  the  moment  when  the 
impression  is  made  and  that  when  the  patient  perceives  it. 

I  should  also  mention  a  kind  of  hyperastliesia  or  rather  of 
âysœstliesla,  in  conséquence  of  which  the  least  stimuli,  such  as  a 
slight  pinch,  the  application  of  a  cold  body,  give  origin  to  a  very 
painful  sensation,  which  is  always  the  same  wbatever  the  nature  of 
the  stimulus  may  be.  According  to  the  statements  of  the  patients,  a 
feeling  of  vibration  is  principally  experienced.  Thèse  vibrations, 
according  to  their  account,  seem  to  ascend  from  the  root  of  the 
limb  at  the  same  time  as  they  descend  towards  its  extremity.  In 
most  cases,  thèse  sensations  persist  during  several  minutes,  some- 
times  a  quarter  of  an  hour  and  even  more,  after  the  cessation  of 
the  exciting  cause  which  determined  them.  The  patient,  under 
such  circumstances,  always  finds  much  difficulty  in  exactly  desig- 
nating  the  place  in  which  the  stimulus  was  applied. 

Finally,  it  is  not  rare  to  find  that  the  stimulation  applied  to  one 
limb,  after  having  producedthe  eftectsjust  mentioned,  is  foUowed  in 
a  little  time  by  an  analogous  sensation  which  seems  to  manifest  itself 
symmetrically  in  a  part  of  the  opposite  limb  corresponding  to  the 

'  Cruveilhier,  '  Anat.  Pathol.,'  No.  xxxviii,  p.  9  ;  M.  ScliifF,  '  Lnlirbucli  der 
Physiol.  des  Menschen,'  1858-59,  p.  249. 


100  NDTEITÎVE    DISORDEES. 

région  wliicli  was  primarily  stimulated.  This  cornes  under  the 
head  and  history  of  wliat  lias  been  denominated  associated  sen- 
sations. 

It  lias  been  souglit,  as  yoii  know,  to  account  in  the  following 
manner  for  the  production  of  the  phenomenon  in  question  : 

When  transmission  of  the  sensory  impressions  in  the  cord  has 
been  rendered  difficult  by  the  interruption  of  a  certain  number 
of  (centripetai)  nerve-tubes^  thèse  impressions  are  supposedly 
transmitted  by  means  of  the  ganglionic  cells,  connected  together 
by  their  prolongations  extending  to  the  uninjured  tubes  :  thèse  im- 
pressions having  reached  the  centre  of  perception  by  this  abnormal 
way  would  be  subsequently  referred,  in  accordance  with  the  common 
rulcj  to  the  j^eriphery  of  thèse  latter  nerve-fibres,  and  hence  would 
supervene  the  error  in  localisation. 

I  believed  it  right,  gentlemen,  to  remind  you  of  thèse  peculiar- 
ities  because,  I  repeat,  they  are  observed  more  commonly  and  in  a 
higher  degree  in  paraplegia  by  compression  than  in  any  other  form 
of  paralysis  of  the  lower  extremities.  But,  again,  we  must  not 
look  hère  for  an  absolute,  distinguishing  characteristic.  Besides, 
this  qualiîication  must  be  remembered,  thèse  symptoms  are  rarely 
observed  in  paraplegia  determined  by  slow  compression  of  the  spinal 
cord,  except  in  cases  where  the  spinal  lésion  is  carried  to  its  highest 
point. 

VI. 

TJnless  unexpected  complications  supervene,  the  nutrition  of  the 
paralysed  parts  remains  normal.  Thus,  the  muscles  préserve,  for 
many  montlis,  their  electric  properties.  Prolonged  inaction,  however, 
finally  brings  about  emaciation  and  diminution  of  the  faradaic 
contractility  of  the  paralysed  muscles.  With  respect  to  the  external 
tégument,  the  bladder  and  the  kidneys,  no  appréciable  nutritive 
change  is  presented,  for  a  long  time.  But,  the  vitality  of  thèse 
organs  appears  to  become  rapidly  modified  under  the  influence  of 
certain  complications.  Thus,  for  instance,  in  a  case  which  I 
observed  of  paralysis  consécutive  on  Pott's  disease,  the  sudden 
opening  of  an  abscess  into  the  rachidian  canal  determined  an  abrupt 
irritation  of  the  inferior  segment  of  the  cord,  which  was  soon  fol- 
lowed  by  the  rapid  formation  of  sacral  eschars,  and  by  an  altération 
in  the  electric  contractility  of  the  muscular  masses  which,  by  degrees, 
became  atrophied  in  a  remarkable  manner.     The  urine,  at  the  same 


SPINAL  HEMIPLEGTA  AND    HEMI-PARAPLEGIA.  101 

time  became  purulent.  The  changes  which  supervene,  under  such 
conditions,  are  generally  speedily  fatal,  Such  altérations,  indeed, 
maj,  without  the  apparent  intervention  of  a  new  cause  or  any 
-complication  whatever,  manifest  themselves  more  or  less  rapidly  at 
a  given  moment,  in  the  course  of  paraplegias  from  compression,  and 
•bring  about  a  fatal  termination. 

VII. 

Hitherto,  gentlemen,  we  hâve  only  occupied  ourselves  with  the 
organic  lésions  which  intercept  the  course  of  the  nerve-fibres  in  the 
cord,  throughout  its  whole  breadth,  in  one  place.  I  wish  now  to  call 
jour  attention  to  a  case  in  which  one  of  the  latéral  halves  of  this 
nerve-centre  is  alone  injured  by  the  compression. 

It  behoves  us  first  to  specify  clearly  the  extent  and  the  disposition 
of  the  altération  with  which  we  are  concerned. 

We  suppose  the  latéral  half  of  the  spinal  cord  afFected  throughout 
its  whole  breadth  to  the  médian  line.  Consequently,  the  lésion 
should  bave  interrupted  the  course  of  the  fibres  of  the  posterior 
and  antero-lateral  columns  of  one  side,  and  simultaneously  also  the 
corresponding  parts  of  the  grey  substance  to  the  médian  line.  In 
thèse  spécial  conditions  and  in  thèse  only,  the  lésion  whose  efFects 
we  are  studying  is  clinically  represented  by  a  most  remarkable  and 
thoroughly  characteristic  symptomatic  group. 

We  may  designate  this  group  simply  by  the  name  of  spinal 
Jiemijplegia  with  crossed  anœsthesia,  when  the  lésion  in  question 
occupies  a  part  of  the  cervical  région. 

If,  on  the  contrary,  it  is  a  portion  of  the  dorsal  région  which  is 
affected  in  this  manner,  it  is  net  hemiplegia  which  is  observed,  but 
spinal  ]iemi-^)araplegia  with  crossed  anastliesia.  You  will  soon 
miderstand^  gentlemen,  the  reason  of  thèse  dénominations. 

VIII. 

Any  liemilateral  lésion  of  the  cord  which  fails  to  fulfil  the 
specified  conditions  just  enumerated  will  not  produce  the  sympto- 
matic group  to  which  I  désire  to  call  your  attention,  or  will  only 
produce  it,  at  most,  in  an  imperfect  manner;  on  the  contrary,  once 
thèse  conditions  are  fulfilled,  the  symptomatic  picture  is  necessarily 
presented.  Our  knowledge  of  this  subject  is  of  quite  récent  date. 
It  is  one  of  the  most  précise,  and  most  fruitful  of  the  results  fur- 
saished  in  later  times  by  the  intervention  of  expérimental  physiology 


102  SPINAL    HEML-PARAPLEGIA. 

in  the  domain  of  spinal  pathology,  and  I  am  happy  lo  say  tliat  this 
important  resuit  is  entirely  due  to  tlie  works  of  my  friend  Professer 
Brown-Séquard.  Not,  indeed,  that  spinal  hemiplegia  and  hemi-para- 
plegia  had  passed  unperceived  until  histime,  but^  before  M.  Browu- 
Séquardj  it  was  thought  that  thèse  formed,  as  it  were^  a  fortuite  us 
reunion  of  strange  and  contradictory  phenomena,  inexplicable  from 
the  standing  point  of  the  prevailing  physiology.  To-day,  thanks 
to  the  labours  of  M.  Brown-Séquard,  we  know,  at  least  to  a  great 
extent,  the  reason  of  the  phenomena  which  we  can  trace  back, 
with  accuracy,  to  the  anatomical  lésion  which  gave  them  birth. 

Por  a  long  time,  clinically  speaking,  the  subject  chiefly  interested 
surgery,  for  a  hemilateral  section  of  the  cord,  capable  of  determin- 
ing  hemi-paraplegia  with  crossed  ansesthesia,  appears  to  be  a  fré- 
quent conséquence  of  injuries  done  to  the  spinal  centre  by  a 
cutting  weapon.  However,  the  physician  is  sometimes  called  to 
observe  this  symptomatic  form  and,  particularly,  in  cases  of  spinal 
compression  occasioned  by  a  tumour. 

Let  us  take  the  case  of  a  meningeal  tumour,  compressiug,  to- 
wards  the  middle  of  the  dorsal  région,  one  latéral  half  of  the  spinal 
cord,  and  suppose,  in  order  to  realise  the  question  more  clearly, 
that  the  compression  aflfects,  for  instance,  the  left  side  of  the  nerve- 
column,  as  happened  in  a  case  whose  history  I  hâve  related  (figs.  6 
and  7).^  Hère  are  the  principal  phenomena  which  would  be 
noticeable  under  such  circumstances  : 

The  inferior  extremity  011  the  left  side  would  be  more  or  less 

'  Charcot,  "  Hémiparaplégie  détennmée  par  une  tumeur  qui  comprimait  la 
moitié  gauche  de  la  moelle  épinière,"  ia  '  Archives  de  Physiologie,'  t.  ii,  p. 
29,  1869,  pi.  viii.  The  tuniour,  beariiig  ou  the  anterior  face  of  the  dorsal 
région  of  the  cord,  which  it  strongly  compresses  from  before  backwards  and 
frora  left  to  right,  is  rather  regularly  oval  in  shape.  Its  long  vertical  axis 
measures  about  three  centimètres  and  a  half,  and  its  transverse  diameter  one 
and  a  half  (figs.  6  and  7a).  It  is  situated  five  centimètres  above  an  imaginary 
Une,  which  would  divide  across  the  widest  part  of  the  lumbar  enlargemeut. 
It  is  partialiy  uiclied  in  a  dépression  which  it  lias  hoUowcd  out  for  itself  at  the 
expcnse  of  the  cord  (tig.  7,  à).  It  is  not  situated  exactly  on  the  médian  Une,  but 
a  little  lo  the  left  of  the  anterior  médian  furrow,  which  it  bas  driven  towards  the 
right,  so  that  it  compresses  the  left  half  much  more  strongly  than  the  right.  In 
one  point,  the  compression  of  the  lei't  half  is  pushed  so  far  that  the  two  layers 
of  the  pia  mater  appear  adhèrent  together  ;  on  the  contrary,  the  right  half  of 
the  cord  still  exhibits,  in  the  most  strongly  compressed  points,  that  is,  in  the 
vicinity  of  the  médian  furrow,  a  thickness  of  more  than  two  millimètres.  See 
also  a  case  published  in  the  '  Lancet,'  1856,  p.  406,  by  IMr.  Ogle. 


CROSSED  HEMI-AN^STHESIA. 


103 


completely  paralysed,  as  regards  movement  ;  the  same  thing  also 
would  occur,  witli  respect  to  the  abdominal  muscles  of  the  same 
side.     The  integuments,  on  the  points  corresponding  to  the  parts 


IlG.    7. 


FiG.  8. 


affected  by  motor  paralysis  présent,  when  compared  with  homolo- 
gous  points  on  the  opposite  side,  a  more  or  less  marked  élévation 
of  température,  as  a  conséquence  of  vaso-motor  paralysis.  Sensa- 
tion, over  the  whole  extent  of  thèse  same  points,  would  be  found 
normal  or  even  remarkably  augmented  on  a  level  with  the  seat  of 
the  spinal  compression  and,  on  the  same  side,  an  attentive  explora- 
tion would  detect  the  existence  of  a  zone  of  anœstliesïa^  disposed  in 


101.  CROSSED    HEMI-AN/f;STHESIA. 

a  transverse  direction,  and  forming  the  upper  boundary  of  tlie 
paralysed  parts,  the  sensibility  of  which,  as  lias  been  stated,  would 
be  exaggerated  or  normal. 

On  the  rïght,  that  is  to  say,  on  tlie  side  opposed  to  the  spinal 
lésion,  the  power  of  movenient  would  be  perfectly  preservod  in  the 
îower  extremity,  and  the  muscles  of  the  abdomen  ;  but,  on  the  other 
hand,  sensibility  would  be  obscured  or  eveu  completely  extin- 
guished,  in  ail  its  kinds.  We  should  hâve  a  geruiine  hemi-anaes- 
thesia,  bounded  above  on  a  level  witli  the  spinal  lésion  by  a  well 
•defined  horizontal  line,  and  bounded  very  exactlrin  front  by  the 
médian  line. 

You  readily  comprehend,  from  what  précèdes,  the  reason  of  the 
îiame  spinal  hemi-paraplegia  with  crossecl  ancpsthesia,  proposed  as  a 
désignation  for  the  symptomatic  group  in  questiori.  If  the  hemi- 
lateral  compression,  instead  of  occupying  the  dorsal  région  of  the 
cord,  should  occupy  a  point  higher  up,  the  superior  portion  of  the 
brachial  enlargement  for  instance,  we  would  hâve  the  symptoms  of 
spinal  hemipleg'ia  j^roperli/  so-called,  before  our  eyes.  Ilere  also 
we  should  notice  a  crossed  hemi-ansesthesia,  one  occupying,  that  is 
to  say,  the  side  of  the  body  opposite  to  that  in  which  the  spinal 
lésion  is  situated  ;  but  the  insensibility  would  not  remain  confined 
to  the  Iower  extremity  and  one  side  of  the  abdomen,  it  would 
extend  on  the  same  side  over  the  trunk,  the  upper  extremity,  and 
even  the  neck,  so  that  the  face,  perhaps,  might  alone  be  spared. 

On  the  side  corresponding  to  the  seat  of  the  spinal  lésion,  the 
motor  paralysis  would  occupy,  at  the  same  time,  the  upper  and 
Iower  extremities,  which  would  botli  présent  a  relative  increase  of 
température.  .  The  trunk  and  the  extremities  of  this  side  would 
hâve  ])reserved  their  sensibility  or  become  bypersesthetic.  The 
zone  of  anassthesia  which  would  form  the  upper  limit  of  thèse  parts 
would  be  situated  very  high,  and  would  occupy,  for  instance,  the 
upper  portion  of  the  thorax,  and  shoulder,  and  even  of  the  neck. 

The  ansesthesia,  exactly  or,  as  it  were,  geometrically  limited,  by 
the  médian  line,  and  extended,  as  we  hâve  described,  over  one  entire 
side  of  the  body,  recalls,  in  some  respects,  the  hemi-anœsthesia  of 
îiysterical  patients,  and  that  which  is  observed,  as  we  hâve  else- 
"where  mentioned,  in  the  case  of  certain  circumscribed  (focal)  lésions 
of  the  encephalon.^     But,  many  circumstances  may  be  noticed   to 

'  Charcot,  'Lectures  on  Diseases  of  the  Neivous  Syslem,'  first  séries, 
Xiecture  X,  New  Sydenliam  Society. 


CROSSBD  HEMI-AN.ESTHESIA.  105 

serve  to  distinguisli  them,  if  need  be.  Thus,  in  hysteria,  as  in  the 
case  of  an  encephalic  lésion,  the  face  would  almost  necessarily  par- 
ticipate  in  the  hemi-anaesthesia,  which  would  not  happen  in  spinal 
hemiplegia,  Besides,  the  concomitant  motor  disorders — paresis, 
paralysis  with  or  vvithout  contracture — would  show  themselves  in 
the  latter  case,  on  the  side  opposed  to  the  anEesthesia,  whilst  they 
would  occupy  the  same  side  as  the  latter  in  hysterical  patients,  and 
in  those  afFected  by  organic  lésions  of  the  encephalon.  I  will  not 
dwell  longer  on  the  subject  of  thèse  distinguishing  détails  which 
might  be  easily  multiplied. 

Nor  will  I  stay  to  develop  further  the  anatomic  and  physiolo- 
gical  interprétation  which  has  been  given  of  the  symptoms  of  spinal 
hemiplegia  and  paraplegia.  I  cannot  do  better,  in  this  respect,  than 
direct  y  ou  to  the  différent  works  of  M.  Brown-Séquard,^  and  I  shall 
confine  myself  to  the  folio wing  remarks. 

It  is  supposed  that  the  conductors  of  sensory  impressions,  whatever 
they  may  be,  after  having  followed  in  each  latéral  half  of  the  spinal 
cord  a  course  tending  from  without  inwards,  and  froin  behind  for- 
wards,  on  a  plane  slightly  iuclining  upwards,  arrive  and  cross  each 
other  at  the  médian  line.  There  is  also  reason  to  believe  that  the 
fascicles,  which  after  this  crossing  ascend  towards  the  encephalon,  do 
not  greatly  diverge  from  the  médian  antero-posterior  plane,  and 
that  they  occupy  the  central  part  of  the  grey  substance  in  the 
vicinity  of  the  commissure.  Now,  hère  are  the  conséquences  which 
resuit  from  such  an  arrangement. 

The  hemilateral  lésion  of  the  spinal  cord  (whether  it  be  caused  by 
a  wound  given  with  a  weapon,  by  a  focus  of  myelitis,  or  by  a 
tumour,  matters  little)  will  hâve  the  effect  of  destroying  a  number 
of  yet  uncrossed  conductors  which  will  be  great  in  proportion  to  its 
vertical  extent.  In  this  way  is  that  transverse  zone  of  ansesthesia 
produced,  more  or  less  high-placed  according  to  the  case,  which  is 
observed  on  the  same  side  as  the  lésion,  and  on  a  level  with  it. 

Below  the  lésion,  the  conductors  coming  from  the  same  side  of 
the  cord  will  foUow  their  course  to  the  médian  line,  and  there  cross 
with  those  of  the  opposite  side,  without  having  undergone  any 
interruption  in  their  path.  Hence  it  is  that  the  parts  situated 
below  the  transverse  zone  of  ansesthesia  will  préserve  their  normal 

'  See  Brown-Séquard,  '  Physiology  and  Pathology  of  tlie  Nervous  System,' 
Philadelphia,  1860.  'Journal  de  la  Physiologie,'  &c.,  t.  vi,  1863,  p.  124. 
*  The  Lancet,'  1869,  vol.  i. 


106  SECONDARY    LESIONS. 

sensibility.     They  verj   often  even  show   theraselves   remarkably 
hyperaesthetic. 

There  bas  not  yet  been  given,  so  far  as  I  am  aware,  a  perfectly 
satisfactory  explanation  of  the  latter  phenomenon. 

Witb  respect  to  the  conductors  of  sensory  impressions,  which 
corne  from  the  side  of  the  cord  opposed  to  that  occupied  by  the 
lésion,  they  hâve  ail,  after  crossing,  to  traverse  the  focus  of  altéra- 
tion provided  that  the  latter  really  extend  to  the  médian  line  ;  and 
they  cousequently  ail  uudergo,  in  this  part  of  their  course,  a  more  or 
less  complète  interruption.  Thus  it  is  that  the  crossed  hemi- 
angesthesia  is  produced. 

As  regards  the  motor  paralysis  which  is  observed  below  the 
hemilateral  lésion  of  the  cord,  and  on  the  same  side  with  it,  that  is 
an  easily  foreseen  conséquence  of  the  interruption  undergone  by 
the  corresponding  latéral  fascicle,  the  fibres  of  which  do  not 
decussate  anywhere  in  the  cord  with  the  homologous  fibres  of  the 
opposite  side. 

I  took  care,  just  now,  to  point  out  to  y  ou  that  the  fascicles — 
supposing  that  it  be  really  the  fascicles — resulting  from  the  decus- 
sation  of  the  conductors  of  sensory  impressions  appear  not  to 
diverge  markedly  from  the  antero-posterior  médian  plane,  where 
they  occupy,  on  eitlier  side,  the  central  part  of  the  grey  substance. 
It  follows  from  this  arrangement  that  a  hemilateral  lésion  of  the 
cord,  even  though  somewhat  severe,  but  which,  not  rigorously 
extending  to  the  médian  line,  spares  the  fascicles  in  question,  would 
not  hâve  the  effect  of  determining  crossed  hemi-ansesthesia.  Such 
a  lésion  would  produce,  according  to  the  case,  spinal  hemiplegia 
or  hemi-paraplegia,  but  without  crossed  ansesthesia.  You  will 
meet  in  practice,  with  rather  numerous  examples  of  this  kind. 

I  hâve  desired  to  restrict  myself,  gentlemen,  to  indicating  to  you 
in  a  very  summary  manner  the  most  salient  features  of  the  sym- 
ptomatic  group  which  betrays  the  existence  of  hemilateral  lésions 
of  the  spinal  cord.  I  canuot,  however,  excuse  myself  from 
adding  to  what  précèdes  some  complementary  détails.  Rarely  do 
the  lésions  in  question  remain  always  confined  within  their  original 
boundaries.  Soon  or  late  they  propagate  themselves,  either 
upwards  or  downwards,  or  in  both  directions  at  the  same  time,  to 
a  certain  distance  beyond  the  primary  focus.  It  is  almost  the 
rule,  for  instance,  that,  below  the  hemilateral  transverse  lésion  and  on 
the  same  side  with  it,  the  latéral  fascicle  shall  be,  at  a  given 


i 


HBMI-PAEAPLEGIA    AND    CAUSES.  107 

moment,  affected  throughout  its  whole  height,  according  to  the  lavr 
of  the  development  of  descending  fasciculated  scléroses.  In  such 
a  case,  permanent  contracture  would  not  delay  to  becoine  super- 
added  to  the  paralysis  determined  in  the  members  by  the  primary 
spinal  lésion.  At  other  times,  the  irritation  seems  also  to  propa- 
gate  itself,  likewise  below  the  lésion  and  on  the  same  side,  so  as 
to  invade  the  anterior  horn  of  grey  matter.  ïhen  the  extremities, 
already  paralysed  and  contractured  would,  in  addition,  exhibit  a  more 
or  less  well-marked  atrophy  of  the  muscular  mass.  Finally,  ap])ar- 
ently  in  connection  with  the  consécutive  altération  of  dilferent 
points  of  the  grey  substance,  not  yet  determined,  we  may  see  the 
symptoms  of  spinal  hemiplegia  become  complicated  by  the  forma- 
tion of  différent  other  trophic  disorders,  such  as  arthropathies,  sacral 
or  gluteal  eschars,  &c. 

Enough  has  been  said,  I  hope,  gentlemen,  to  make  you  recognise 
the  interest  which,  from  our  point  of  view,  belongs  to  the  study  of 
spinal  hemiplegia.  I  cannot  too  often  repeat  that  this  symptomatic 
array  is  not  produced  only,  as  for  a  time  was  thought,  in  consé- 
quence of  traumatic  lésions  of  the  spinal  cord.  I  hâve  myself 
observed  it,  under  the  well-defined  form  of  hemi-paraplegia  with 
crossed  ansesthesia  in  five  cases.  In  three  of  thèse  cases,  there  was 
transverse  scierons  myelitis  ;  in  a  fourth,  an  intra-spiual  neoplasia.i 
Lastly,  in  the  fifth,  already  meutioned  above,  a  tumour  primarily 
developed  in  the  internai  surface  of  the  dura  mater  had,  at  a 
given  moment,  by  enlarging,  in  an  antero-posterior  direction,  deter- 
mined in  one  part  of  the  dorsal  région  a  compression  which  was 
almost  exactly  confined  to  one  latéral  half  of  the  spinal  cord. 

^  Charcot  et  Gombault,  "Note  sur  uu  cas  de  lésions  disséminées  des  centres 
nerveux  observées  chez  une  femme  syphilitique,"  iu  '  Archives  de  Physio* 
logie,'  1873,  p.  173. 


LECTURE   VIII. 

ON  SLOW  COMPRESSION  OF  THE  SPINAL  CORD.  CERVICAL 
PARAPLEGIA,  PECULIAR  SYMPTOMS.  PERMANENT  SLOW 
PULSE. 

■SuMMARY. — O71  cervical  paraplegia.  Coiiijjression  of  nerves  of 
upper  extremities.  Lésions  of  spinal  corcl  in  tlie  nech  ;  their 
mode  of  action  in  tlie  production  of  cervical  paraplegia.  Dis- 
tinction hetîveen  cervical  paraplegia  due  to  the  comjiression  of 
periplieral  nerves,  and  that  dépendant  on  a  lésion  of  the  cord. 
Altération  qf  motor  nerve-cells  and  tumours  of  the  cord — 
causes  of  a  thirdform  of  cervical  paraplegia. 

On  some  peculiar  symptoms  of  slow  compression  of  the  cer- 
vical cord.  Oculo-pupillary  disorders.  Cough  and  dijspnœa. 
Frequentli/  recurring  voniiting.  Bifficulti/  of  déglutition. 
Hiccough.  Fimctional  dérangement  of  hladder.  Epileptic 
attacJcs. 

On  p)ermanent  sloio  puise.  Temporari/  slowing  of  puise  in 
fractures  qf  vertelrœ  qf  nech.  Permanent  sloio  puise  con- 
nected  with  certain  organic  affections  of  heart  [aortic  in- 
sufficiency,  fatty  degeneration  of  cardiac  iissue,  fihrinous 
deposits).  Insufficiency  of  cardiac  lésions,  in  some  cases,  to 
explain  slow  puise.  Phenomena  connected  with  permanent 
slow  puise.  Syncope,  apojdectiform  state,  convulsive  fits .  In 
certain  cases,  the  starting  point  of  permaneyit  slow  puise  to  he 
sought  in  cervical  cord  or  bulbus  rachidicus.  Dr.  Halberton's 
case. 

Sudden  death  from  rupture  of  transverse  ligament  of  the 
odontoid  process. 

Symptoms  accompanying  lésions  of  the  lumhar  enïargement 
and  cauda  equina. 

'Gentlemen, — I  sliall  conclude  tliis  séries  of  studies  relating  to 


CERVICAL  PARAPLEGIA.  109 

slow    spinal    compression,   by  calling   your    attention  to    certain 

peculiaritics  wliich    are   sometimes    observed    in    cases  where  the 

lésion  which  détermines  the  phenomena  of  compression  affects  the 
cervico-brachial  région  of  the  spinal  cord. 


I. 

We  will  consider,  in  the  first  place,  a  singular  form  of  paralysis 
which  may  be  met  with,  under  such  circumstances,  and  which  we, 
following  the  example  of  Dr.  Gull,  propose  to  designate  by  the  very 
practical  name  of  cervical paraplegia}  The  paralysis  hère  occupies 
either  but  one  of  the  upper  extremities,  or  both  of  them  together, 
exclusively  or  at  least  in  a  predominating  manner.  The  abdominal 
extremities  are  also  often  subsequently  affected,  but  generally  in  a 
comparatively  minor  degree.  It  is  not  very  uncommon  to  meet 
this  form  of  paralysis  in  Pott's  disease  of  the  cervical  région. 

a.  It  may  happen  and,  in  fact,  it  somewhat  frequently  occurs 
that,  in  this  variety  of  Pott's  disease,  the  nerves  of  the  upper 
extremities  are  compressed,  sometimes  at  the  intervertébral  notches, 
sometimes  in  their  passage  through  the  dura  mater,  where  it  is 
thickened  by  a  caseous  pachymeningitis.  This  compression,  when 
carried  to  a  certain  degree,  will  necessarily  sooner  or  later  resuit  in 
the  production  of  a  paralysis  affecting,  according  to  the  case,  either 
both  the  upper  extremities  simultaneously  or  one  of  them  singly, 
This  paralysis  is  wholly  peripheral,  and  will  be  represented,  gentle- 
men, by  the  following  train  of  symptoms  nearly  : 

At  the  beginning,  acute  pain  will  occupy  the  course  of  the  com- 
pressed and  irritated  nerve-trunks  :  a  more  or  less  marked  hyper- 
sesthesia  of  the  integuments  may  be  added,  together  with  various 
vaso-motor  disorders,  différent  vesicular  and  bullar  cutaneous  érup- 
tions, &c.,  in  a  Word — the  whole  séries  of  phenomena  which  we  but 
lately  learned  to  know  in  référence  to  the  pseiido-neuralgias  of  the 
cord,  and  to  which,  consequently,  it  is  needless  for  us  to  return. 
Loss  of  motor  power  will  not  fail  to  supervene  without  delay,  and 
the  muscles  of  the  paralysed  members  will  soon  présent  a  more  or 
less  well-marked  atrophy,  usually  accompauied  by  a  greater  or  less 
degree  of  decrease  of  faradaic  contractility  in  the  course  of  the 
disease.  The  hypersesthesia  will  give  place  to  ansesthesia  which  is 
often  very  intense.     Lastly,  it  shoukl  be  remarked  that,  in  the 

'  "Cervical  Paraplegia,"  '  Guj's  Hospital  Reports,'  t.  v,  1858,  p.  207, 


110  CEEVTOAL  PARAPLEGIA. 

members  deprived  of  motion,  diminution  or  even  total  suppression 
of  tlie  reflex  acts  may  supervene.^ 

h.  The  compression  undergone  by  the  nerve-filaments  which  give 
origin  to  the  brachial  plexus,  is  far  from  being  the  only  organic 
cause  capable  of  producing  cervical  paraplegia  ;  the  latter  may  still 
show  itself  in  conséquence  of  lésions  which  act  upon  the  spinal 
cord  itself. 

If  it  is  true  that  very  severe  compression,  carried  so  far^  for 
instance,  as  to  flatten  the  cord  in  a  marked  manner,  bas  necessarily 
the  efFect,  when  bearing  on  the  cervico-brachial  région,  of  paraly- 
sing  the  four  extremities,  it  is,  on  the  other  liand,  demonstrated  by 
observation  that  a  lesser  pressure,  affecting  the  same  région,  may 
under  certain  conditions  resuit  in  occasioning  a  motor  paralysis 
confined,  for  some  time  at  least,  to  the  two  upper  extremities  or 
even  to  one  of  them. 

In  order  to  account  anatomically  and  physiologically  for  this 
phenomenon,  which  clinical  observation  bas  set  prominently  forth, 
it  bas  been  proposed  to  admit  that  the  conductors  for  the  volun- 
tary  motor  incitations  of  the  thoracic  members  occupy,  in  the 
autei'ior  columns  of  the  cervical  cord,  a  more  superficial  plane  than 
that  occupied  by  the  conductors  of  the  same  incitations  for  the 
inferior  extremities.  It  foUows  naturally  from  this  that  the  two 
orders  of  conductors  may  be  separately  and  singly  injured." 

Whatever  be  the  value  of  this  interprétation,  there  can  be  no 
doubt,  gentlemen,  I  repeat,  as  to  the  existence  of  the  form  of  para- 
lysis of  the  upper  extremities  to  which  I  bave  to  call  your  atten- 
tion. Hère,  besides,  is  a  summary  statement  of  the  peculiar 
features  under  which  it  présents  itself,  which  allow  it  to  be  dis- 
criminated  from  the  cervical  paralyses  connected  with  peripheral 
compression  of  the  nerves.  In  the  latter,  as  you  know,  the  loss  of 
motor  power  is  accompanied  and  preceded  by  acnte  pains  (pseudo- 

I  "  On  Cervical  Paraplegia  from  Compression  of  Nerve-trunks,"  see  Brodie  ; 
"Injuries  of  the  Spinal  Cord,"  in  '  Medico-Cliirurgical  Transactions,'  1837,  t. 
20,  p.  131;  Marshall  Hall,  in  '  Medico-Chir.  Trans.,'  1839,  *•  22,  p.  216; 
Niemeyer,  '  Speciell.  Pathol.,'  t.  ii,  p.  358;  Prof.  Rosenthal,  '  Canstatt's 
Jahresbericht,'  1866,  2  Bd.  i  abth.,  p.  45,  and  '  Nervenkrankh.;'  Benedikt, 
'Elektrotherapie,'  t.  ii,  p.  316  ;  J.  A.  Michaud,  '  Sur  la  méningite  et  sur  la 
mvclite  dans  le  mal  vertébral,'  Paris,  187 1,  p.  56. 

-■  See  Brown-Séquard,  'Journal  de  la  Physiologie,'  &c.,  t.  vi,  1865,  p.  139, 
631,  and  632.     Eulenburgh,  '  Functionellen  Nervenkrankh.,'    Berlin,    1871^ 

P-  379- 


CERVICAL  PARAPLEGIA.  1]] 

neuralgias),  followed  sooner  or  later  by  ansesthesia,  a  more  or  less 
well-marked  atrophy  of  tlie  muscular  raass  supervenes  with  j^reater 
or  lesser  rapidity,  together  with  diminution  of  faradaic  reaction. 
The  reflex  acts  in  the  paralysed  members  are  diminished  or  sup- 
pressed. 

Cervical  paraplegia  arising  from  antero-posterior  spinal  compres- 
sion, on  the  contrary,,  présents  itself  with  quite  other  characters. 
Hère,  the  muscles  for  a  length  of  time  préserve  their  volume  as  weil 
as  their  electrical  properties.  The  sensibility  of  the  paralysed  mem- 
bers may  net  be  markedly  modified  ;  lastiy,  not  only  do  the  reflex 
phenomena  persist,  in  thèse  members,  but  they  even  show  them- 
selves  sometimes  manifestly  exalted.  This  circumstance  which,  of 
itself,  would  suffice  to  demonstrate  that  the  lésion  does  not  bear  on 
the  peripheral  course  of  the  nerves  has  ah-eady  been  pointed  out,  in 
a  case  recorded  by  Dr.  Budd,  which  forms  part  of  a  very  interest- 
ing  memoir,  the  publication  of  which  goes  back  to  the  year  1839.^ 
The  patient  was  a  scrophulous  young  girl,  afFected  with  caries  of 
the  cervical  vertcbrse,  in  whom  a  retro-pharyngeal  abscess  was 
subsequently  developed.  Por  nearly  two  years  the  paralysis  was 
confined  to  the  upper  extremities  ;  it  afterwards  invaded  her  right 
lower  extremity.  Under  the  influence  of  différent  stimuli,  as  like- 
wise  during  the  acts  of  micturition  and  défécation,  more  or  less 
energetic  movements  supervened  in  this  limb,  and  also  occasionally 
but  always  to  a  minor  degree,  in  the  upper  extremity  of  the  same 
side. 

A  case  recorded  by  Dr.  Radcliffe  must  be  approximated,  in 
certain  respects,  to  that  of  Dr.  Budd.- 

c.  There  is  also,  gentlemen,  another  method  by  which  an  altéra- 
tion of  the  cervico-brachial  région  of  the  spinal  cord  may  déter- 
mine a  motor  paralysis  limited  to  the  upper  extremities.  You  are 
aware  that,  in  certain  cases  of  infantile   spinal  paralysis  cervical 

1  "Pathology  of  the  Spinal  Cord,"  ia  'Med.-Chir.  Trans.,'  1839,  t.  22,  p. 
141. 

"  A  case  noted  in  Oppoizer's  '  Clinique  '  by  Dr.  Schott,  and  anotlier  case 
observed  by  JEH.  Vogel  and  Ditmar,  are  also  examples  of  slow  compression, 
bearing  on  the  anterior  part  of  the  cervical  région  of  the  spinal  cord,  and 
liaving  determined  a  motor  paralysis  limited  to  the  upper  extremities.  Thèse 
two  cases  hâve  been  referred  to  in  an  interesting  work  by  Dr.  Emil.  Rollet, 
"  Krankheits-Erscheinungen  in  Folge  von  Compression  der  oberster  Dorsal- 
sliicks  des  Ruckenmarks,"  in  '  Wiener  Med.  Wochenschr.,'  Nos.  24,  25,  26, 
and  '  Canstatt's  Jahresb.,'  1865,  t.  3,  p.  30. 


112  PECULTAE  SYMPTOMS. 

paraplegia,  is  observée!  when  tlie  systematic  lésion  of  the  an- 
terior  cornua  of  grey  matter  remains  confinée!,  for  a  certain 
height,  to  tlie  brachial  enlargement.  The  paralysée!  meœbers 
then  présent,  froin  the  outset,  an  extrême  flaccidity,  tlie  resuit 
of  loss  of  tone  by  the  muscles  ;  the  reflex  acts  are  more  or 
less  completely  abolished,  and  the  wasted  muscles  answer  no 
longer  to  the  faradaic  stimulus.  Sensibility,  as  a  gênerai  rule,  is 
changed  in  nothing. 

An  acute  partial  myelitis,  having  the  same  position,  would 
produce  nearly  the  same  eftects/  and  the  same  may  be  said  of  a 
tumour  which,  primarily  developed  towards  the  centre  of  the 
cervical  cord,  sliould  principally  extend  forward  so  as  to  affect 
chieily  the  grey  substance  of  the  anterior  cornua.  Only  in  the 
latter  case  the  aspect  of  the  symptoms  would  be  necessarily  more  or 
less  profoundly  modified,  on  account  of  the  relatively  slow  évolu- 
tion of  the  lésion  and  of  its  extension,  inévitable  as  it  were  at  a 
given  moment,  either  to  the  white  columns  or  to  the  posterior 
régions  of  the  grey  substance.  However  this  may  be,  we  might 
quote  some  instances  where  a  cervical  intra-spinal  tumour  deter- 
mined  a  paralysis  in  the  upper  extremities  which,  in  many  respects, 
approximated  to  the  infantile  type.  In  référence  to  this  matter,  I 
shall  confine  myself  to  pointing  out  a  case,  recorded  by  Dr.  GuU, 
where  the  patient  was  a  child  of  eight  raonths,  in  whom  a  solitary 
tubercle  had  developed  at  the  inferior  portion  of  the  cervical  en- 
largement,  on  a  level  with  the  origin  of  the  sixth  and  seventh 
cervical  nerves.  The  paralysis  at  first  gradually  invaded  the  right 
superior  extremity  ;  then,  at  the  end  of  fifteen  days,  it  had  ex- 
tended  to  the  left  upper  extremity.  Two  months  after  the  invasion 
of  the  first  symptoms,  the  paralysed  members  which  were  greatly 
wasted,  hung  flaccid  and  powerless  on  either  side.  The  abdominal 
members  were  weak,  but  the  little  patient  could  move  tliem  volun- 
tarily.  Death  supervened  six  months  after  the  appearance  of  the 
paralysis.  Up  to  the  last  moment,  voluntary  movements  persisted 
to  a  certain  extent  in  the  lower  extremities." 

I  will  dwell  no  longer  hère  upon  this  last  variety  of  cervical 
paralysis  of  spinal  origin  ;  it  shall,  besides,  form  the  subject  of  a 
detailed  study. 

^  'OUivier  d'Anj^er,'  t.  il,  p.  319,  3e  édition. 

-  '  Guy's  Hospital  Reports,'  1858,  p.  206,  Case  32.  See  also  Case  15  of 
the  same  worli,  1856,  p.  181,  wliere  the  cause  was  probably  a  glioma. 


I 


OCULO-rUPlLLARY    SYMPTOMS.  113 

II. 

I  propose  now  to  offer  some  remarks  relative  to  a  certain  number 
of  sjmptoms  which  are  occasionally  manifested  in  conséquence  of 
lésions  bj  compression  either  of  the  cervical  région  or  of  the  upper 
portion  of  the  dorsal  région  of  the  cord. 

The  symptoms  in  question  deserve  ail  the  more  to  detain  us 
because^  on  the  one  hand,  they  bave  hitherto,  generally  speaking 
at  leastj  been  little  remarked  ;  and  because,  on  the  other  hand,  they 
may  for  several  weeks  or  longer  exist  in  a  state  of  isolation,  tbat  is 
to  say,  independent  of  ail  motor  paralysis  of  the  limbs,  thus  con- 
stituting  as  it  were,  during  that  time,  the  only  clinical  révélation  of 
the  spinal  disease. 

a.  You  are  not  unaware  that  more  or  less  marked  oculo-^upillary 
disorders  are  rather  frequently  the  product  of  traumatic  lésions,  bear- 
ing  on  the  cervical  or  superior  dorsal  cord.  Sometimes  we  find  dilata- 
tion {m//osisspastica),  sometimes,  on  the  contrary,  contraction  of  the 
pupil  {înt/osis  parali/tica)  jWnà^i  such  circumstances  ;  they  occasion- 
ally affect  but  one  eye,  occasionally  both  are  taken  at  the  same 
time.  In  the  same  eye,  the  two  orders  of  phenomena  may  occur 
in  succession,  and  then  spasmodic  dilatation  précèdes  paralytic  con- 
traction.^ Thèse  facts,  at  the  présent  day,  form  part  of  the 
common  stock  of  knowledge.^  But  what  is  perhaps  less  knowi» 
is  the  fact  that  mydriasis,  resulting  from  permanent  irritation  of 
the  cilio-spinal  région  determined  by  a  traumatic  cause,  may  snbsist 
continuously,  during  several  weeks,  without  the  addition  of  paralysis 
of  the  extremities,  as  was  demonstrated  by  a  case  recorded  by 
Dr.  Eosenthal,  to  vv'hich  I  will  shortly  return. 

Sirailar  modifications  of  the  pupil  may  be  shown  in  connexion 
with  lésions  by  compression  of  the  superior  régions  of  the  cord, 
Mr.  Ogle  bas  referred  to  their  existence  in  several  cases  of  cervical 
Pott's  disease.  In  a  case  of  the  same  kind,  published  by  Dr.  A. 
Eulenburg,^  the  pupil  remained  very  evidently  dilated  for  four 
weeks,  after  which  it  gradually  resumed  its  normal  dimensions. 

^  Gerhardt,  '  Centralblatt,'  1865,  p.  10. 

^  Leudet,  'Mena,  de  la  Société  de  Biologie,'  1863,  p.  105.  Rendu,  "Des 
troubles  fonctionnels  du  grand  sympathique  observés  dans  les  plaies  de  la 
moelle  cervicale."  In  'Archives  Géu.  de  Méd.,'  Sept.,  1S69,  pp.  286 — 297. 
A.  Eulenburg  und  P.  Guttraann,  'Pathologie  des  Sjmpathicus/  Berlin,  1873, 
p.  9. 

'  A.  Eulenburg,  '  Greifswalder  Med.  Beitràge,*  1864,  iii,  pp.  81,  88. 

VOL.  II.  8 


114  COUGH  :    DYSPNŒA. 

Tlie  osseous  afTection  in  tliis  patient  seemed  to  occupy  tlie  last 
•cervical  vertebra  and  tlie  fîrst  tliree  dorsal  vertébrée.  A  case  reported 
by  Dr.  E.  Rollet,  from  Oppolzer's  clinique;,^  is  peculiarly  inter- 
€sting  as  regards  the  question  at  issue,  because  it  shows  dilatation 
of  both  pupils,  accompanied  by  a  certain  degree  of  protrusion  of 
the  ocular  globes,  preceding  by  some  time  the  development  of  motor 
paralysis  in  the  lower  extremities.  The  cause  was  the  existence  of 
tuberculosis,  occupying  the  tliird  and  fourth  dorsal  vertebrae,  which 
h  ad  determined  by  compression  the  softening  of  the  anterior 
columns  in  the  corresponding  région  of  the  spinal  cord.  It  would, 
no  doubtj  be  easy  to  multiply  examples  of  the  same  nature. 

h.  I  would,  in  the  second  place,  notice  in  a  very  particular 
manner  the  cough  and  the  dyspnœa  which,  in  compression  of  the 
upper  régions  of  the  spinal  cord,  may  exist,  as  isolated  symptoms, 
long  before  the  appearance  of  paraplegia.  When  combined  with 
the  neuralgic  pains,  which,  in  such  a  case,  naturally  occupy  the 
superior  part  of  the  thorax,  thèse  symptoms  hâve  sometimes  repro- 
duced  the  semblance  of  incipient  phthisis,  so  closely  as  to  render 
mistakes  easy  ;  this  is  a  circumstance  to  which  the  practical  sensé 
of  Dr.  Gull  has  not  failed  to  give  suitable  prominence,  and,  with 
référence  to  this,  lie  mentions  a  case  whicli  I  thiuk  it  useful  you 
should  know,  at  least  in  a  summary  manner. 

The  patient  was  a  baker,  aged  thirty  years,  who  at  the  time  of  ad- 
mission to  Guy's  Hospital,  hadbeen  suffering  for  about  two  months 
from  cough  and  dyspnœa,  accompanied  by  pains  in  the  upper  part 
of  the  back,  as  well  as  in  the  right  shoulder,  by  fréquent  perspira- 
tions,  by  a  certain  degree  of  wasting,  and,  lastly,  by  prostration. 
Tour  days  after  his  entrance  he  suddenly  found  himself  unable  to 
pass  water  voluntarily,  and  fifteen  days  later  his  knees  became  pain- 
ful  (?  spinal  arthropatliies),  whilst  at  the  same  time  the  thoracic  pain 
increased  ;  then  the  power  of  movement  began  to  diminish  in  the 
lower  extremities.  Motor  paralysis  progressively  supervened  in  thèse 
members,  and  soon  showed  itself  complète  and  thorough.  Sensi- 
bility  was  also  naturally  lessened  in  the  paralysed  members  and  in 
the  whole  of  the  lower  portion  of  the  trunk,  to  a  level  with  the 
third  rib.  A  vast  bedsore  having  supervened  in  the  sacral  région 
the  patient  succumbed  about  four  months  after  the  invasion  of  the 
first  disorders.  The  spinal  cord  was  found  softened,  on  examination, 
l'or  about  an  inch  in  extent,  throughout  its  whole  breadth,  at  the 

'  Loc.  cit.,  'Caustatt's  Jahresb./  1863,  t.  iii,  p.  30. 


FREQUENT   VOMITING.  115 

first  dorsal  vertebra.  A  tumour  as  big  as  a  filbert  was  attaclied  to  the 
inner  surface  of  the  dura  mater  ;  it  had  determined  the  compression 
of  the  cord,  from  before  backwards,  on  a  level  with  the  softened 
point.  The  lower  lobes  of  both  lungs  presented  the  lésions  of 
récent  pneumonia  ;  no  trace  of  any  anterior  lésion  could  be  dis- 
covered  in  thèse  organs.i 

Symptoms,  analogous  in  every  respect  to  those  which  hâve  just  been 
mentioned,  are  likewise  found  in  another  case,  also  recorded  by  Dr. 
Gull,  in  which  the  spinal  affection  was  not  the  resuit  of  compression. 
It  was  constituted  by  an  induration  which  occupied  the  cervical 
enlargement.^ 

c.  Varions  gastric  disorders,  and  particularly  oft-recurnng 
vomiting,  should  hâve  a  place  among  the  phenomena  which  are 
sometimes  connected  with  the  first  symptoms  of  cervical  spinal  com- 
pression. This  symptom  showed  itself  very  remarkably  in  a  case 
where  the  affection  was  due  to  an  intra-spinal  tumour  (probably  a 
glioma),  which  occupied  the  central  part  of  the  cord  in  the  lowet 
half  of  the  cervical  enlargement."  It  existed  also  in  the  little 
patient  mentioned  above/  who  suffered  from  solitary  tubercle, 
developed  in  the  same  région.  It  is  proper  to  put  thèse  digestive 
dérangements  on  a  parallel  with  the  gastric  crises  of  locomotor 
ataxia  and  gênerai  spinal  paralysis;^  but  it  is  especially  important, 
from  the  standing  point  of  pathological  physiology,  to  point  out 
tliat  very  tenacious,  very  persistent  vomiting  is,  apart  from  ail 
cérébral  commotion,  a  rather  fréquent  immédiate  symptom  in  con- 
nection with  spinal  lésions  occasioned  by  fracture  of  the  cervical 
vertébrée.  This  fact  lias  been  already  noticed,  though  in  a  passing 
way,  by  Brodie  ;  but  it  has  been  decidedly  demonstrated  by  the  inter- 
esting  statistics  of  Dr.  Gurlt,  based  upon  the  analysis  of  300  cases 
of  fracture  of  the  cervical  vertébrée  occurring  in  différent  régions.^ 

1  W.  Gull,  '  Guy's  Hospital  Reports,'  3rd  séries,  t.  ii,  1856,  Case  i,  p.  145. 
-  Same  collection.  Case  16,  p.  185. 
^  Gull,  loc.  cit.,  t.  ii,  p.  184,  Case  15. 

*  Gull,  loc.  cit.,  t.  iv,  p.  206,  Case  32. 

*  Charcot,  '  Lectures  on  Diseases  of  the  Nervous  System,'  2nd  séries, 
cil.  viii. 

*  E.  Gurlt,  '  Handb.  der  Lchre  von  deu  Knochenbrûchen,'  2tli  i  Lief,  1864, 
p.  62.  In  one  of  Brodie's  cases  the  vomited  fluid  was  of  a  blackish  colour. 
The  mucous  membrane  of  the  stomach  was  mottled  with  ecchymotic  patches  ; 
and  the  cavity  of  the  organ  was  filled  with  a  fluid  like  cofFee  dregs,  in  one  of 
the  cases  recorded  by  Gurlt  (No.  35). 


116        DIFFICULTE    OF    DEGLUTITION.      EPILEPTIC    FITS. 

d.  Difficidti/  ofdeghtHion,  of  more  or  less  intensity  and  persistence, 
and  Jnccough,  may  be  approximated  to  the  gastric  dérangements  in 
question.  They  supervene  in  the  same  circumstances^  and,  in  cer- 
tain cases  of  compression  of  the  cervical  cord,  sometimes  make 
their  appearance  long  beforethat  of  the  paralysis  of  the  extremitiesJ 
The  same  may  be  said  with  respect  to  the  functional  disorders  of  the 
hladder  and  rectum^  and  the  latter  fact  contrasts  remarkably  with 
what  we  hâve  learned  with  respect  to  the  behaviour  of  thèse  organs, 
when  the  compression  bears  on  the  dorsal  cord.  This  is  a  point 
which  it  was  of  interest  to  elucidate. 

e.  I  shall  merely  mention  the  epileptic  attacks  which  sometimes 
show  themselves^  in  a  periodic  manner,  in  patients  suffering  under 
spinal  lésions  from  compression.  Contrary  to  what  might  hâve  been 
supposed  from  the  well-known  effects  of  sections  of  half  the  spinal 
cord,  in  certain  animais,  epilepsy  in  man  appears  to  be  compara- 
tively  rather  a  rare  resuit  of  spinal  lésions.  However,  I  hâve  been 
able  to  collect,  with  care,  ten  cases  of  this  kind,  about  half  of 
which  relate  to  lésions  of  the  cervical  cord  determined  by 
compression. 

The  most  remarkable  of  thèse  cases  is  undoubtedly  that  which 
was  published  in  1862,  in  the  *  Gazette  des  Hôpitaux,'  by  M. 
Duménil,  of  Rouen.^  You  will  not  confound  thèse  gênerai  con- 
vulsions of  spinal  origin  with  the  symptomatic  group  described  by 
M.  Brown-Séquard  under  the  name  of  spinal  epilepsy,  to  which  we 
hâve  several  times  already  called  your  attention  in  the  course  of 
thèse  lectures.^     The  convulsions,  whether  tonic    or    clonic,   are 

1  Gull,  loc.  cit.,  Cases  15,  32. 

2  Gull,  loc.  cit.,  Cases  i,  15,  16, 

=*  A.  Duménil,  loc.  cit.,  p.  478.  See  also  the  observations  of  Geddings,  of 
Baltimore.  (Brown-Scquard,  '  Journal  de  la  Physiologie,'  t.  vi,  p.  633)  ;  of 
Webster  (-Medico-Chirurgical  Traiis.,'  2nd  séries,  t.  viii),  of  Gendrin  ('Ollivier 
d'Angers,  t.  ii,  pp.  502  and  520),  of  Charcot  and  Bouchard  (Bouchard,  "Des 
dégénérations  secondaires  de  la  moelle  épinière"),  extract  from  the  '  Archives 
Générales  de  Médecine,'  1866,  p.  32  ;  in  the  latter  case  the  compression  bore 
rather  npon  the  bulbus.  As  regards  epilepsy  in  connection  with  lésions  of  the 
dorsal  and  lumbar  régions  of  the  spinal  cord,  see  Leudet  ('Archives  de  Méd.,' 
1863,  t.  i,  p.  266),  '  Ollivier,  d'Angers,'  3e  édition  (1837,  t.  ii,  p.  319)  ;  Rilliet 
et  Barthez  (t.  iii,  p.  5S9,  1859);  Michaud  (' Sur  la  méningite  et  la  myélite,' 
Paris,  1871,  p.  50)  ;  Brown-Séquard  ('  Researches  on  Epilepsy,'  p.  11)  ;  West- 
phal  ('Archives  de  Psychiatrie,'  t.  i,  p.  84,  1868);  'Ollivier,  d'Angers,'  (t.  ii, 

p.  319)- 

<  Charcot,  '  Lectures   on   Diseases   of   the  Nervous   System,'    ist  séries 

pp.  201,  202. 


KBTAEDATION    OF    PULSE.  117 

iimited,  in  the  later  cases,  you  know,  to  tlie  parts  situated  below 
the  lésion  of  the  spinal  cord. 

f.  One  of  the  most  interesting,  but  also,  if  I  do  net  mistake,  one 
of  the  least  noticed  facts  of  the  symptomatology  of  cervical  spinal 
lésions  is^  beyond  contradiction,  the  permanent  retardation  of  the 
puise,  whicli  is  sometimes  found  as  a  conséquence  of  thèse  lésions. 

Surgical  observation  has  long  since  recognised  that  fractures  of 
the  cervical  vertébrée  hâve  rather  frequently  the  effect  of  giving  rise 
to  a  remarkable  slowness  in  the  pulsations  of  the  heart.  Such,  in 
particular,  is  the  resuit  of  fractures  affecting  the  5th  and  6th 
cervical  vertebrse.  In  such  circumstances,  Mr.  Hutchinson  has  seen 
the  puise — which  always,  according  to  him,  remains  regular,  con- 
trary  to  what  occurs  in  case  of  cérébral  shock' — give  only  forty- 
eight  beats  per  minute.^  According  to  Dr.  Gurlt,  whose  important 
statistics  I  hâve  already  commended  to  your  notice,  the  pulsations 
may  even  descend  to  thirty-six  and  to  twenty.  Fractures  of  the  Ist 
dorsal  appear  to  be  of  themselves  capable  of  inducing  slowness  of 
the  puise."  It  is  understood  that  ail  interférence  from  cérébral  con- 
cussion is  eliminated  in  thèse  cases.  As  a  rule,  slowness  of  the 
puise  in  connection  with  fractures  of  the  cervical  région  is  an  essen- 
tially  transitory  phenomenon,  and  soon  gives  place  to  a  very  marked 
accélération,  which  is  almost  always  a  bad  omen. 

It  sometimes  happens,  however,  that  it  persists  as  a  permanent 
symptom  for  several  weeks.  In  référence  to  this,  I  would  revert 
to  the  case  of  Dr.  Eosenthal,  of  Vienna,  mentioned  above.  A  child 
of  fifteen  years  received  a  blow,  which  struck  it  in  the  région  of  the 
sixth  cervical  vertebra.  Symptoms  of  a  slight  and  quite  transitory 
cérébral  shock  immediately  showed  themselves,  accompanied  by 
hemiplegia  of  the  riglit  side,  which  did  not  last  more  than  twenty- 
four  hours.  Nevertheless,  during  the  four  weeks  following  the 
accident,  besides  the  dilatation  of  the  pupil,  already  mentioned, 
it  was  remark  ed  that  the  number  of  heart-beats  remained 
permanently  lowered  to  a  very  notable  extent.  The  pulsations 
oscillated  between  56  and  48  per  minute.  The  patient  completely 
recovered. 

'  Hutchinson,  "  On  Fractures  of  the  Spine,"  '  London  Hospital  Reports,' 
1866,  t.  iii,  p.  366. 

'  Gurlt,  loc.  cit.,  p.  50,  obs.  61,  borrowed  from  Hughes  (' Dublin  Hosp. 
Eeports,'  t.  ii,  1855,  p.  145),  and  obs.  22  reported  by  Tyrrel  ('London  Med- 
and  Phys.  Journal,'  t.  61,  new  séries,  vol.  vi,  1829,  p.  232). 


118  SLOW  PULSE.       IRRITATIVE   SPINAL    LESIONS. 

Does  not  this  undoubtedly  most  remarkable  case  render  it  already 
highly  probable  that  the  phenomenon  ai  permanent  slow  puise  may^ 
in  certain  circumstances,  be  observed,  with  ail  its  conséquences,  after 
irritative  lésions  of  the  cervical  cord,  apart  even  from  ail  traumatic 
influence  ?  I  say  "  with  ail  its  conséquences,"  because  in  reality, 
as  you  will  acknowledge  in  a  moment,  permanent  slow  puise  is  far 
from  being  an  indiffèrent  phenomenon,  if  it  but  happen  to  be  very 
distinctly  marked. 

Apart  from  traumatic  lésions  of  the  cervical  cord  or  medulla 
oblongata,  slow  puise,  in  the  opinion  of  the  few  authors  who  hâve 
studied  it,  is  only  observed  as  a  conséquence  of  certain  organic  dis- 
eases  of  the  heart — aortic  insufficiency,  fatty  degeneration  of  the 
ventricular  muscles,^  or  the  présence  of  fibrinous  deposits  (?  infarc- 
tus) in  their  tissues.^  I  am  far  from  wishing  to  deny  that  the  pheno- 
menon of  slow  puise  may,  in  fact,  hâve  its  starting  point  in  an  organic 
lésion  of  the  heart.  But  I  must  déclare  that  I  hâve  three  times 
observed  this  persistent  phenomenon,  in  a  very  marked  form  (20, 
30  beats  a  minute),  established  permanently,  for  several  years^  in 
aged  inmates  of  this  refuge  ;  and  that,  in  thèse  three  cases,  after  an 
attentive  anatomical  vérification,  the  heart  was  found  either  quite' 
healthy,  or  merely  presenting  changes  of  a  most  common-place  kind, 
especially  at  such  a  period  of  life.^  Hence,  I  hâve  been  induced  to 
ask  myself  whether,  at  least  in  cases  where  cardiac  lésions  are 
absent,  the  organic  cause  of  the  slowness  of  arterial  pulsations 
would  not  réside  in  the  cervical  cord  or  medulla  oblongata  rather 
than  in  the  heart.  It  is  true  that  the  anatomical  investigations 
which  I  undertook  in  référence  to  this  subject  hâve  remained 
till  now  without  any  definite  resuit.  But  it  is  important  to 
observe  that  they  date  from  a  period  when  our  means  of  explora- 
tion, in  ail  that  relates  to  the  nerve-centres,  were  much  less  power- 
ful  than  they  hâve  since  become. 

^  W.  Stokes,  "  Observations  on  some  cases  of  permanenllj  slow  puise  " 
(' Dublia  Quarterly  Journal  of  Médical  Science,'  Aug.  i,  1846).  '  Traité  djps 
maladies  du  cœur  et  de  l'aorte,'  trad.  par  le  Dr.  Sénac,  pp.  138,  332,  308,  315, 
337.     R.  Quain,  *  Medico-Chir.  Trans.,'  t.  xxxiii. 

*  Ogle,  "  Fibrinous  masses  deposited  in  the  substance  of  the  heart's  walls  ; 
remarkable  slowness  of  the  puise  ;  epileptic  seizures  "  ('  Pathological  Society,' 
1863,  p.  89. 

^  ïhe  heart,  on  auscultation  and  percussion,  did  not  exhibit  any  sigu  of  altér- 
ation in  a  very  interesting  case  of  permane7it  slow  puise  with  fainting  and  epi- 
leptic fits,  published  by  M.  A.  Rotureau,  in  '  l'Union  Médicale  '  (ler  Mars^ 
1870,  No.  25,  p.  331).    See  also  Note  B. 


CONSECUTIVE   PHENOMENA.  119 

If  I  insist,  gentlemen,  on  permanent  slow  puise  considered  in  its 
possible  relations  with  spinal  or  bulbar  lésions,  it  is  not  only 
because  this  phenomenon  is  one  the  interprétation  of  which  is  of  the 
highest  interest  to  pathological  physiology,  but  also  because,  as  I 
gave  you  to  understand,  just  now,  that  serious  phenomena  are  very 
usually  superadded,  which  are  capable  of  occasioning  death,  with 
rapidity.  Thèse  phenomena  are  thus  constituted.  They  supervene 
in  paroxysms,  recurring  irregularly  at  more  or  less  remote  periods  | 
sometimes  they  appear  with  ail  the  characters  of  syncope  ;  sorae- 
times,  as  regards  the  symptoms,  they  partake  of  the  nature  of  those 
of  syncope  and  of  the  apoplectic  state  ;  there  are  finally  cases  in  which 
epileptiform  movements  are  superadded,  which  are  especially  notice- 
able  in  the  face,  with  change  of  colour,  foaming  at  the  mouth,  &c. 
The  puise,  which,  in  the  interval  of  the  crises,  beats  on  an  average 
30  or  40  times  a  minute,  becomes  still  slower  during  the  fit,  and 
may  descend  to  20,  or  even  to  15  pulsations.  It  may  even  stop 
for  a  moment,  and  sometimes  altogether.  The  state  of  syncope 
always  opens  the  scène  ;  the  apoplectic  state  with  stertorous  sleep 
then  supervenes,  at  the  moment  when  the  puise,  which  had  been 
for  an  instant  suppressed,  reappears,  and  when  the  pallor  of  the 
features  gives  place  to  redness  of  the  countenance.  The  epilepti- 
form convulsions  which  occasionally  appear,  supervene  under  the 
self  same  conditions. 

The  array  of  symptoms  remains  invariable,  gentlemen,  as  my 
three  observations  demonstrate,  whether  there  be  organic  lésions  of 
the  heart,  well  and  duly  attested,  or  whether  thèse  lésions  be  non- 
existent.  What  then  is  the  origin  of  the  slowness  of  the  jiulse  and 
of  the  superadded  phenomena,  in  cases  of  the  last  kind  ?  I  am 
much  inclined  to  believe,  I  repeat,  that  it  should  be  sought  for  in 
the  spinal  cord  or  meduUa  oblongata.  In  the  absence  of  per- 
sonal  observations  adapted  to  décide  the  question,  I  can  base  my 
hypothesis  not  only  on  what  has  been  just  said  on  the  effects  pro- 
duced  by  traumatic  irritation  of  the  superior  régions  of  the  cord, 
but  also  on  the  knowledge  of  a  peculiar  fact,  which  is  extremely 
remarkable,  thougli  it  has  hitherto,  I  know  not  wherefore,  re- 
mained  in  the  shade. 

The  case  belongs  to  Dr.  Halberton,  who  published  it  in  the 
^  Medico- Chirurgical  Transactions,^  of  London,  for  1844.^  The 
patient  was  a  gentleman,  aged  64,  who  fell  upon  his  head,  whilst 

'  ï.  II.  Halberton,  "  A  case  of  slow  puise  witli  fainting  fîts,  which  first  came 


120  SPINAL    AND    BULBAR   LESIONS. 

out  liunting,  and  lost  consciousness  at  once.  He  was  obligecl  to 
keep  his  bed  several  weeks,  complaining  of  acute  pain  in  the  neck, 
and  much  difficulty  in  moving  his  head.  This  difficulty  persisted 
for  a  long  time  ;  however,  during  the  two  years  subséquent  to  the 
accident,  he  could  follow  most  of  his  favourite  occupations,  with 
more  or  less  ease.  Not  until  the  end  of  thèse  two  years,  did  the 
first  syncopal  crisis,  a  fainting  fit,  supervene  ;  and  it  was  then 
ïioticed  for  the  first  time  that  the  puise  had  become  permanently 
slow.  During  the  subséquent  two  or  three  years,  the  paroxysms 
were  repeated,  and  at  shorter  and  shorter  intervais,  whilst  the  fits 
became  longer.  Generally,  in  thèse  crises  the  syncopal  state  soon 
gave  place  to  the  apoplectiform  and  epileptiform  phenomena  which 
ï  hâve  mentioned.  The  puise  which  under  ordinary  conditions 
gave  an  average  of  33,  fell  to  20,  and  even  to  15  at  the  approach 
of  the  attack,  and  it  ceased  for  a  moment  to  beat  when  this  had  set 
in. 

Death  supervened  in  one  of  thèse  crises,  and  the  following  signs 
were  observed  at  the  autopsy,  which  was  made  by  Lister.  The 
upper  portion  of  the  spinal  canal,  and  the  occipital  foramen  were 
considerably  narrowed  in  the  antero-posterior  direction,  so  that  the 
little  finger  could  scarcely  be  passed  into  the  latter  orifice.  The 
dura  mater  and  the  ligament  which  covers  the  posterior  part  of  the 
body  of  the  axis,  were  very  much  thickened.  The  atlas  preserved 
its  normal  position,  but  the  articulations  which  unité  it  to  the 
occipital  bone  had  undergone  osseous  anchylosis,  so  that  no  motion 
was  possible.  The  meduUa  oblongata  was  very  small  and  very 
firm.  The  heart  was  large,  the  ventricle  walls  rather  thin,  but 
apart  from  a  certain  degree  of  thickness  of  the  endocardium  in 
several  cavities,  it  presented  no  altération  worthy  of  notice. 

The  author  does  not  hesitate  to  attach  ail  the  symptoms  noted 
in  his  interesting  observation — permanent  slow  puise,  fainting  fits 
followed  by  apoplectiform  and  epileptiform  symptoms — to  the 
effects  of  the  compression  undergone  by  the  cervical  cord  and 
medulla  oblongata,  owing  to  the  diminution  of  calibre  presented  by 
the  vertébral  canal  and  occipital  foramen.  I  endorse  his  opinion 
without  réserve.^ 

ou  two  years  after  an  injury  of  the  neck,  from  a  fall"  ('  Med.-Chir.  Traus.,  ' 
t.  24,  London,  1841). 

'  Permanent  dow  puise  with  faintiug,  apoplectiform  aud  epileptiform  lits  are 
aometimes  seen  as  sequelse  of  diphtheria.     Tliere  is  reason  to  believe,  from 


LESIONS    OF    LUMBAR   ENLARGEMENT.  121 

This  is  the  place  to  remind  you  of  the  terrible  accident  wliich 
somewliat  frequently  happens  in  Pott's  disease  of  the  cervical 
région  ;  I  refer  to  the  rupture  of  the  transverse  ligament  which 
maintains  the  odontoid  process  on  the  axis,  and  the  dislocation  of 
the  process  which  is  the  conséquence.  The  history  of  the  efFects  of 
the  abrupt  compression  of  the  cervical  cord  and  medulla  oblongata 
which  then  supervenes  is  not  adapted  for  long  descriptive  détails  ; 
it  is  sudden  death, — death  sans  phra&e, — if  I  may  use  the  word, — 
which  results.  This  accident,  I  repeat,  is  far  from  rare.  Mr.  Ogle 
has,  himself  alone,  coUected  four  cases  of  this  kind  in  the  course 
of  his  hospital  work.^ 

III. 

To  finish  with  ail  that  relates  to  the  history  of  slow  spinal  com- 
pression, it  remains  for  me  to  say  a  few  words  in  référence  to  the 
particular  symptoms  which  are  met  with  when  the  lésion  affects 
the  lumbar  enlargement  or  the  cauda  equina.  I  will  be  brief  on 
this  point,  because  it  lias  not  yet  been,  so  far  as  I  know,  the  object 
of  sufficient  clinical  research.  The  only  facts  to  be  noticed  with 
regard  to  the  occurrence  of  profound  altérations  occupying  the 
lumbar  enlargement  throughout  the  whole  of  its  inferior  position 
to  the  filum  terminale  are  :  the  flaccidity  of  the  paralysed  limbs, 
marked  inertia  of  the  anal  and  vesical  sphincters,  and  diminution 
or  even  suppression  of  reflex  acts."  If  the  lésion  should  be  situate 
on  one  side  only  of  the  enlargement,  let  us  say  on  the  right  side, 
and,  for  instance,  on  a  level  with  the  third  sacral  pair  of  nerves, 
extending  a  little  above  and  below  this  point,  the  following  pheno- 
mena  would  be  observed  :  right  motor  paralysis  afFecting  little  more 

■what  précèdes,  that  thèse  symptoms,  which  writershave  always  sought  to  attach 
either  to  an  altération  of  the  ventricle  walls,  or  to  the  formation  of  clots  in 
the  cardiac  cavities,  arise  in  certain  cases,  at  least,  from  a  lésion  afFecting  the 
medulla  oblongata  or  the  superior  cervical  cord;  this  is  a  thesis  the  discussion 
of  which  I  reserve  for  auother  opportunity.  See  Milner  Barry,  'British 
Médical  Journal,'  July,  185S;  R.  Thompson,  '  Médical  Times,' Jan.,  1860; 
Eisenmann,  "  Der  rersache  der  diphtherischen  Làhmungen"  ('Deutsche  Klinik.,' 
Juli,  1861,  No.  29,  p.  286)  ;  Greenhow,  '  Clinical  Society  of  Loudon  '  ('The 
Laucet,'  May  4,  1872,  p.  615). 

'  Ogle,  '  Pathological  Society,'  1863,  p.  17. 

2  Brown-Séquard,  '  Diagnostic  et  traitement  des  principales  formes  de  para- 
lysie des  membres  inférieurs,'  Paris,  1864,  p.  73.  W.  Ogle, 'Pathological 
Society,'  1853,  t. iv.  'Fracture  of  the  last  dorsal  vertebra  with  destruction  of 
the  spinal  marrow.' 


123  LESIONS  OF  CAUDA  EQUINA. 

than  the  leg  and  foot  ;  préservation  of  the  sensé  of  feeling  on  this 
side  in  the  paralysed  parts;  complète  or  nearly  complète  anses- 
thesia  of  the  corresponding  parts  of  the  left  side  with  rétention  of 
voluntary  movement.  We  should  also  find, — and  this  it  is  which 
enables  us  to  clifFerentiate  such  a  case  from  those  where  the  hemi- 
lateral  lésion  is  seated  higher  in  the  cord, — loss  of  sensibility  in 
différent  parts  of  both  sides  of  the  trunk  and  inferior  members, 
especially  at  the  anus,  the  perinaeum,  and  the  knees.^ 

The  conséquences  of  compression  of  the  nerves  of  the  cauda 
equina,  naturally  belong  to  the  history  of  lésions  of  peripheral 
nerves.  The  pseudo-neuralgic  pains,  motor  paralysis,  and  ansesthesia 
would  necessarily  vary  in  position  and  extent,  and  that  according 
to  the  mode  of  distribution  and  the  degree  of  lésion  of  thèse  nerves, 
The  sphincters  of  the  anus  and  of  the  bladder  should^  in  such  a 
case,  remain  generally  intact,  but  bed-sores  of  rapid  development 
might  form  in  the  sacral  région  and  on  other  parts  of  the  lower 
extremities.^ 

Hère,  gentlemen,  will  terminate  our  discourse  touching  the  sym- 
ptoms  of  spinal  compressions.  If  time  had  permitted,  I  should  hâve 
been  glad  to  show  you,  by  the  examination  of  particular  instances, 
what  advantage  we  can  dérive  from  a  knowledge  of  the  facts 
which  we  hâve  registered,  in  the  clinical  observation  of  diseases  of 
the  spinal  cord.  To  my  great  regret,  I  find  myself  coerced  to  leave, 
as  a  project  for  the  future,  this  labour  of  applied  investigation. 

'  See,  in  référence  to  this,  the  very  interestiug-  observation,  though  not 
followed  by  autopsy,  reported  by  M.  Brown-Séquard  iu  the  '  Journal  de  la 
Physiologie,'  t.  vi,  1863,  p.  624,  obs.  xxiii. 

2  Brown-Séquard,  loc.  cit.,  p.  623  ;  Knapp  ('  New  York  Journal  of  Medicine,' 
Sept.,  1851,  p.  198);  Desruelles,  'Société  Anat.,'  1852,  p.  12;  '  London 
Hospital  Reports,'  t.  iii,  1866,  p.  343). 


PART    THIRD. 


ON    SPINAL   AMYOTROPHIES. 

INFANTILE  SPINAL  PARALYSIS  ;  ADULT  SPINAL  PARALYSIS  ; 
SPINAL  PROGRESSIVE  MUSCULAR  ATROPHY  ;  AMYOTRO- 
PHIC  LATERAL  SCLEROSIS,  &c. 


LECTURE  IX. 

INFANTILE  PARALYSIS.i 

SuMMARY. — Spinal  myopai/iies  or  myopathies  qf  spinal  origin. 
General  characters.  Localisation  of  spinal  lésions  in  the 
anterior  cornua  of  grey  matter. 

Infantile  spinal  par aly  sis. — To  he  considered  as  a  disease  for 
study.  Symptoms. — Period  of  invasion.  Us  modes  ;  second 
period  or  retrogression  qf  symptoms  loith  localisation  of  muscular 
lésions  {muscular  atrophy,  arrest  of  development  of  the  osseous 
System,  coldness  qf  extremities,  deformities,  paralytic  cluh- 
foot). 

Pathological  anatomy  of  infantile  paralysis. — Lésions  of 
muscles  at  différent  periods  ;  fatty  deposits.  Lésions  of  the 
nervous  system  ;  history  {Charcot  and  Cornil,  Vulpian  and 
Prévost,  Charcot  and  Joffroy ,  Par  rot,  LocTchart  Clarke  and  John- 
son, Bamaschino  and  Roger).  Localisation  of  lésions  in  the 
anterior  cornua  of  grey  substance.  Secondary  altérations  ; 
scierons  transformation  of  neuroglia  ;  foci  of  disintegration  ; 
partial  sclerosis  of  the  antero-lateral  columns  ;  atrophy  of 
the  anterior  roots.  Jteasons  tending  to  demonstrate  that  the 
primary  lésion  résides  in  the  nerve-cells. 

Gentlemen, — Allow  me  to  call  your  attention  to  a  nosograpbical 
group,  wliich  I  propose  to  designate  by  the  name  of  spinal  myo- 
pathies or  myopathies  qf  spinal  origin. 

Atrophie  lésion  of  the  muscles^  varying  in  extent  and  intensity, 
is  a  feature  coramon  to  ail  individuals  of  the  group,  and,  in  fact, 
constitutes  their  most  salient  clinical  characteristic. 

Again,  the  muscular  affections  in  question  appear  to  be  always 

^  This  lecture,  delivered  at  La  Salpêtrière,  in  July,  1870,  was  published  in 
'La  Revue  photographique  des  Hôpitaux,'  in  January  and  February,  1872. 


126        GEEY  SUBSTANCE  OF  SPINAL  COED. 

-correlated  with  an  altération  which,  in  a  predominating,  if  not 
exclusive,  manner,  occupies  certain  well-deterrained  éléments  of 
the  grey  substance,  namely,  the  apparatus  of  nerve-cells  termecl 
motor,  which,  as  you  are  aware,  are  situated  in  the  anterior  cornua 
of  the  grey  substance  of  the  spinal  cord. 

Before  entering  upon  a  spécial  study  of  the  différent  affections 
which  constitute  this  group,  suffer  me  to  give  you  some  preliminary 
détails,  calculated  to  bring  out  the  gênerai  characters  which  I 
désire  to  indicate  in  a  very  summary  manner. 

Although  the  central  grey  substance  occupies  a  comparativelylimited 
space  in  the  spinal  cord,  it  nevertheless  is  the  most  important  part 
of  the  spinal  centre,  viewed  from  a  physiological  stauding-ground. 
It  will  be  enough  for  me  to  remind  you  that  this  central  cord  of  grey 
substance  is  thegangway  for  the  transmission  of  sensory  impressions; 
that  the  volitional  and  reflex  motor  impulses  should  also  necessarily 
pass  through  the  grey  substance— -so  that,  if  this  passage  were  eut, 
the  accomplishment  of  ail  thèse  functions  would  be  simultaneously 
rendered  impossible.  But  it  seems  to  be  now  demonstrated  that 
ail  the  parts  of  the  grey  substance  are  not  indiscriminately  quali- 
fied  for  the  exécution  of  thèse  différent  functions.  In  that  limited, 
that  circumscribed  space,  which  the  grey  substance  occupies  in  the 
centre  of  the  spinal  cord,  we  hâve  reason  to  distiuguish  several 
régions,  several  very  distinct  departments.  Thus  it  is,  for  instance, 
that  M.  Brown-Séquard,  and,  after  him,  M.  Schiff,  séparâtes 
'phydologically,  in  a  very  clear  manner,  what  he  terms  the  central 
grey  matter  from  the  grey  cornua.  The  transmission  of  sensory 
impressions  would  seriously  concern  the  first  mentioned  région 
only  (with,  at  least  partially,  the  posterior  cornua).  As  to  the 
anterior  cornua  they  appear  to  be  destined,  above  ail,  for  the  trans- 
mission of  motor  impulses,  and  to  hâve  little  concern  with  sensi- 
bility. 

Gentlemen,  thèse  results  which  are  founded  upon  physiological 
experiments,  hâve  been  confirmed  by  pathology.  For  disease  some- 
times  détermines  altérations  that  affect,  singly  and  severally,  the 
différent  régions  of  the  grey  substance,  and  that  in  a  manner 
more  satisfactory  thau  the  most  skilful  physiologist  could  effect. 

This  is  precisely  the  case  as  regards  those  affections  which  we 
are  about  to  describe.  They  are  determined  by  a  lésion  which 
may  occupy  exclusively,  or  nearly  so,  the  anterior  cornua  ;  and 
consequently,  whilst  the  transmission  of    sensory   impressions  is 


MOTOR    NERVE    CELLS.  127 

modified  in  notliing,  except  perhaps  in  a  merely  accessory  manner, 
as  if  by  chance,  the  motor  functions  are  on  tlie  contrary  pro- 
foundly  injured. 

This  absence  of  sensory  change  is  a  feature  which  differentiates 
the  diseases  of  the  group  from  the  différent  forms  of  myehtis 
we  shall  soon  hâve  to  study,  and  which,  like  the  preceding,  may 
afïect  the  central  grey  substance. 

In  thèse  central  myélites  the  infiammatory  lésion  bears  indis- 
criminately  on  ail  points,  on  ail  régions  of  the  grey  substance  ; 
whence  it  follows  that  sensibility  and  movement  are,  of  necessity, 
simultaneously  altered.  The  motor  functions  and  muscular  nutri- 
tion are  alone  affected,  on  the  contrary,  in  cases  of  spinal  myo- 
pathies, properly  so  called,  at  least  in  pure  types — types  free  from 
any  complication.  And,  since  we  are  engaged  in  comparing  myelitis 
with  spinal  myopathies,  let  us  also  mention  the  following  characters 
which  belong  to  the  former  and  not  to  the  latter. 

The  muscular  affection  is,  in  the  latter  case,  confined  to  the 
muscles  of  animal  life,  particularly  to  those  of  the  extremities  ;  the 
trunk  and  the  head  are,  indeed,  far  from  being  exempt  ;  but  the 
functions  of  the  bladder  and  rectum  are  generally  respected. 

Contrary  to  what  takes  place  in  ordinary  myelitis,  it  is  also  rare 
to  see  esc/iars  (bed-sores)  or  otlier  disorders  of  the  nutrition  of  the 
skin  in  spinal  myopatides,  even  in  the  most  serions  cases. 

Einally,  the  exaltation  ofreflew  action,  the  différent  forms  of  spinal 
epilepsy  which  are  seen  in  certain  myélites,  and  the  permaneîit  con- 
tracture which  is  superadded, — and  which  also  constitutes  one  of 
the  symptoms  of  scierons  diseases  of  the  white  antero-lateral 
columns  at  a  certain  stage  of  development — ail  thèse  are  absent  in 
cases  of  spinal  myopathies. 

In  short,  gentlemen,  lésions  of  the  muscular  System  of  animal 
life,  whose  présence  is  betrayed  by  motor  impotence  and  by  wasting, 
of  a  more  or  less  marked  degree,  are,  as  I  hâve  indicated,  the  pre- 
dominating  clinical  characteristic  of  the  diseases  which  compose  the 
nosographic  group  which  we  propose  to  study  together.  But,  in 
référence  to  tins,  it  is  proper  to  lay  down  an  important  distinction, 

Sometimes,  motor  impotence  displayed  in  a  certain  number  of 
muscles  or  groups  of  muscles  is  the  first  symptom  which  observa- 
tion detects.  The  muscle  is  first  paralysed  ;  the  motor  functions 
are  more  or  less  completely  annulled  ;  altération  of  the  muscular 
structure  appears  only  to  take  place  in  a  secondary  manner. 


128  SPINAL    MYOPATHIES. 

At  otlier  times,  on  the  contrary,  the  affected  muscles  are,  from. 
the  outset^  the  seat  of  very  marked  trophic  dérangements  ;  and, 
in  such  cases,  tlie  lack  of  motor  power  seems  to  be  in  some  sort 
proportionate  to  the  degree  of  atrophy  which  the  muscles  undergo. 

Thèse  are  two  extrême  types,  connected  together  by  numerous 
intermediate  cases  ;  for  frequently,  most  frequently  perliaps,  the 
stricken  muscles  are  both  paralysed  and  atrophied,  and,  besides, 
more  or  less  completely  altered  in  their  structure. 

The  disorders  which  we  are  about  to  assemble  under  the  same 
heading  hâve  been  hitherto  entirely  separate,  in  nosography,  as 
though  they  were  radically  distinct  diseases.  As  an  example,  it 
may  suffice  to  quota  infantile  spinal  paralysis,  gênerai  spinal 
jparalysis,  recently  described  by  M.  Duchenne  (de  Boulogne),  and 
which  has  not  yet  been  allowed  right  of  domicile  in  the  standard  caté- 
gories, glosso-lahio-laryngeal  paralysis,  certain  forms  oi progressive 
muscidar  atrophy,  &c.  I  trust  to  demonstrate  that  the  bringing 
together  of  thèse  diseases,  which  we  are  about  to  attempt,  will 
render  évident  some  common  characteristics  which,  up  feo  the 
présent,  hâve  been  ignored.^ 

'  AU  the  muscular  atrophies,  developed  under  tlie  influence  of  a  spinal 
lésion  {spi7ial  amyotrophies)  may  be  redueed  to  two  fundamental  groups.  In 
one  group,  the  disease  is  evolved,  anatomically  and  clinically,  in  an  acute  or  evea 
a  superacute  manner.  In  the  other,  it  assumes  in  its  course  the  characters  of  a 
primarily  chronic  disease.     We  hâve  hère  grounds  to  make  a  marked  division. 

The  group  of  rapidly  developed  spinal  amyotrophies,  however  circumscribed 
it  may  be,  already  ofFers  a  tolerably  vast  field  for  study  ;  for  the  lésions  of 
the  spinal  cord  which  may  entail  the  speedy  developmeut  of  muscular  atrophy 
are  many.  We  may  mention,  as  examples,  acute  central  myelitis,  i.  e.  principally 
localised  in  the  grey  substance,  liœmaiomyelia,  différent  forms  of  traumatic 
myelitis,  whether  due  to  sudden  compression  caused  by  displacement  of  a  frac- 
tured  vertebra,  or  arising  from  a  wouud  produced  by  an  instrument  penetrating 
the  rachidian  caual  ;  and,  finally,  infantile  i^raly sis. 

Amongst  thèse  spinal  lésions,  so  différent  in  origin  and  in  nature,  there  is 
one  whose  fundamental  anatomical  characteristic  is  to  connect  itself  systemati- 
cally,  so  to  say,  with  the  régions  of  tl)e  grey  substance  occupied  by  the  great 
motor  cells,  whose  atrophy  and  even  complète  destruction  it  détermines.  This 
affection,  which  is  no  other  than  infantile  paralysis,  constitutes,  consequently, 
in  the  group  of  acute  spinal  amyotrophies,  a  remarkable  type  which  ought, 
first  to  be  examined,  because  the  medullary  lésion  and  its  results  are  hère  pro- 
duced under  conditions  which  are  comparatively  simpler  and  therefore  more 
favourable  for  analysis  than  any where  else.  ('  Cours  d'Anatomie  Pathologique- 
de  la  Taculté,'  Avril,  1874.) 


INTANTILB  PAKALYSIS.  129 


II. 


But  it  is  tiine,  gentlemen,  to  leave  thèse  preliminary  considéra- 
tions, whicli  are  too  gênerai  not  to  be  somewhat  vague,  and  to  enter 
upon  an  analysis  of  the  facts.  We  will  sélect  as  a  standard  the 
singular  disease  whicli  is  commonly  known  by  the  name  of  infantile 
paralysïs.  That  is,  in  fact,  one  of  the  most  remarkable  types  of 
the  group  ;  the  spécifie  cliaracters  are  hère  displayed  in  the  most 
striking  manner  ;  hence,  of  the  kind,  infantile  paralysis  may  be 
presented  as  a  model  disease  for  study  ;  for,  if  we  succeed  in  rightly 
rendering  évident  to  you  the  most  salient  features  of  its  history  the 
task  which  remains  to  be  accomplished  will  hâve  been  made  easy, 
as  I  think  you  will  acknowledge. 

You  are  aware  that  the  disease  in  question  is  one  which,  up  to  a 
certain  point,  pertains  to  childhood.  In  fact,  it  is  most  frequently 
developed  between  the  âges  of  from  one  to  three  years.^  After 
five,  the  cases  are  rare  ;^  and  after  ten,  they  are  altogether  excep- 
tional.^  But  it  is  important  to  observe,  gentlemen,  that  we  may 
find  developed  in  the  aduit,  and  even  at  a  mature  âge,  an  af- 
fection which  difFers  in  nothing  essential  from  infantile  paralysis  ; 
so  that,  side  by  side  with  the  sphial paralysis  ofcJdldhood,  we  hâve 
reason  to  make  a  place  for  spinal  paralysis  of  the  adult.  This  is 
a  point  which  M.  Duchenne  (de  Boulogne)  bas  prominently  set 
ont,  which  other  observers  hâve  recognised  also,*  and  which  I  shall 
note  in  my  turn. 

I  shall  state  in  a  few  words  the  symptoms  which  characterise 
this  affection  ;  and,  for  greater  clearness,  we  will  distinguish,  in  our 
description,  the  existence  of  two  periods. 

First  period. — 1°.  The  mode  of  invasio7i  of  infantile  paralysis  is, 
jou  are  aware,  a  most  remarkable  one.  The  disease  has  an  abrupt, 
sudden  beginning,  generally  ushered  in  by  intense  fever,  either  with 

'  Laborde,  'De  la  paralysie  (dite  essentielle)  de  l'enfauce,'  Paris,  1864, 
p.  98. 

^  Laborde,  loc.  cit,  p.  63.  Heine,  '  Spinale  Kinderlâhmung,'  2c  Aufl,  Stutt- 
gardt,  1860,  p.  60. 

*  Duchenne  (de  Boulogne)  fils,  '  De  la  paralysie  atrophique  graisseuse  de 
l'enfance,'  Paris,  1864,  p.  21. 

*  Duchenne  (de  Boulogne)  'De  l'electrisation  localisée,'  3e  édit.,  1872, 
p.  437  ;  M.  Meyer,  '  Die  Electricitàt  und  ihre  Anwenduug,'  &c.,  Berlin,  1868, 
p.  210  ;  E-oberts,  in  Reynolds'  '  System  of  Medicine,'  p.  169. 

VOL.   II.  9 


130  MODES    OF    INVASION. 

or  without  accompanying  convulsions,  or  other  cérébral  symptoms, 
and  sometimes  transient  contractures. 

This  initial  fever,  io  wliich  we  hâve  just  called  your  attention,  is 
observed,  I  repeat,  in  the  majority  of  children  ;  however,  it  may 
sometimes,  it  would  appear,  be  altogether  absent.^ 

However  this  may  be,  the  paralytic  symptoms  show  themselves 
witli  sudden  completeness  ;  between  day  and  morrow,  and  from 
the  very  outset,  they  hâve  reached  their  summum  in  extent  and 
intensity.  Thèse  paralytic  symptoms  présent  great  diversity  as 
regards  the  parts  affected.  At  times,  the  paralysis  is  absolute, 
complète,  and  aiFects  the  four  extremities,  or  three  of  them  :  again, 
it  may  take  only  one  lower  extremity,  or  even  one  of  the  superior 
extremities  -^  at  other  times,  but  very  rarely  indeed,  it  affects  both 
superior  extremities  f  finally,  there  are  cases  where  the  paralysis, 
attacking  the  inferior  extremities  alone,  assumes  the  paraplégie 
form. 

To  sum  up,  we  observe  hère  a  complète  absolute  paralysis,  with 
flaccidness  of  the  extremities,  and  with  abolition  or  diminution  of 
reiiex  excitability,  but — and  this  is  a  point  ou  which  I  still  lay 
stress — without  any  trace  of  loss  of  sensibility,  of  dermal  nea'osis, 
or  of  functional  dérangement  of  bladder  or  rectum.^ 

Does  pain  or  formication  exist,  at  the  commencement,  which  would 
indicate  at  least  a  temporary  participation  of  the  central  grey  sub- 
stance ?  Some  observations  made  by  MM.  Duchenne  and  Heine, 
in  the  case  of  children  old  enough  to  furnish  information  on  this 
subject,  tend  to  establish  an  affirmative.  The  symptoms  remarked 
in  the  adult,  in  similar  cases,  go  to  support  this  opinion,  as  we  shall 
see  elsewhere.  This,  however,  is  most  frequently  but  a  transitory 
and  incidental  phenomenon,  and,  indeed,  the  absence  of  any  marked 
altération  of  sensibility,  contrasting  with  so  complète  and  absolute 
a  motor  paralysis,  is  one  of  the  most  striking  characters  of  infantile 
paralysis.^ 

Hère  also  is  another  feature.     At  a  period  very  close  to  the  outset 

^  E,.  Volkmann,  "  Ueber  Kinderlâhmung  und  paralytische  coutractureu," 
in  'Samnilunçj  Klinisclier  Vortràge,'  No.  i,  Leipzig,  1870,  pp.  3,  4. 

"  11.  Volkmami,  loc.  cit. 

3  Duchenne  (de  Boulogne)  fils,  loc.  cit.,  pp.  13,  18  ;  L.  Clarke,  '  Med.-Chir. 
Transactions,'  t.  li,  1868. 

^  Volkmann,  loc.  cit.  This  author  remarks  tliat  tlie  sexual  functions,  in  the 
adult,  are  not  interfered  witli. 

^  Duchenne  (de  Boulogne)  loc.  cit.,  Volkniann,  h  c.  cit.,  &c. 


EEGRESSION  OF    SYMPTOMS.  131 

of  the  disease,yaradaic  contractility  is  diminished  in  a  great  number 
of  the  paralysed  muscles,  and  extinguished  in  several  of  them  ;  this 
is  an  important  phenomenon,  which  M.  Duclienne  (de  Boulogne)  bas 
several  times  verified  after  tlie  fifth  day,  but  which  is  more  frequently 
met  with  on  the  seventh  and  eighth  days.  I  may  hère  remind  you  that, 
according  to  some  authors,  galvanic  contractility  may  still  set  in 
action  muscles  which  faradisation  no  longer  affects.  Every  muscle 
which,  after  the  lapse  of  a  few  weeks  from  the  invasion  of  the  dis- 
ease,  does  not  react  is  threatened  with  destruction  for  life.^ 

Such,  gentlemen,  are  the  more  salient  characters  of  the  first  period 
of  infantile  paralysis  ;  I  request  your  permission  to  sum  them  up  in 
a  few  words  : 

1°.  Abrupt  invasion  of  the  motor  paralysis  which,  at  the  first 
blow,  attains  its  summum  of  intensity,  either  subséquent  to  a  more 
or  less  intense  fébrile  state,  or  in  the  absence  of  fever. 

2°.  Prompt  diminution  and  even  apparent  abolition  of  faradaic 
contractility  in  a  certain  number  of  muscles  smitten  with  paralysis. 

3°.  Absence  of  marked  disturbance  of  sensibility — of  paralysis 
of  rectum  orbladder;  absence  of  eschars  or  other  cutaneous  trophic 
disorders. 

Second  period. — Gentlemen,  régression  of  the  symptoins  we 
bave  just  discussed  inaugurâtes  the  second  period  of  infantile 
paralysis.  It  begins  to  show  itself  from  the  second  to  the  sixth 
month  after  the  invasion;  sometimes  sooner,  sometimes  later.  It 
takes  several  raonths  to  complète  its  work — six  months,  in  some 
cases,  according  to  Volkmann.  Eight  or  ten  months  after  the  inva- 
sion,— an  epoch  which  marks  the  close  of  this  rétrograde  period, — 
the  muscles  which  bave  not  recovered  their  functions  may,  according 
to  most  observers,  be  considered  as  for  ever  injured,  as  irreparably 
lost.  On  the  other  hand,  the  improvement  which  may  take  place 
does  not,  as  a  gênerai  rule,  show  itself  in  every  point.  In  ordinary 
cases,  there  are  always  some  muscles,  occasioually  those  of  an  entire 
extremity,  or  only  those  of  a  particular  région  of  a  member,  in 
which  the  lésions  continue,  on  the  contrary,  to  make  progress  for  a 
certain  time  still;  thenthey  persist  indelibly,  and  offer  to  the  observer 
a  séries  of  phenomena  which  deserve  to  detain  us  for  spécial  study. 

*  Volkmatm,  '  Klin.  Vortrage,'  p.  5.  Ou  the  other  hand,  judgiug  from  my 
own  expérience,  I  find  that,  occasioually,  muscles  which  hâve  long  ceased  to 
react,  may  regain  their  faradaic  contractility  and  recover  their  functions,  more 
or  less  completely. — G.  Sigerson. 


132  ATROPHY.      ARTÎEST    OF   DEVELOPMENT. 

a.  Atrophy  soon  becomes  manifest  in  those   muscles  in  wliich 
J'araclaic  contractilïty  lias  not  reappeared.     One  does  not  always 

correctly  ajiprehend  the  extent  of  this  atrophy,  because  it  is  often 
naasked,  we  niust  remember,  by  an  accumulation  of  cellulo- fatty 
tissue.  It  constitutes,  nevertheless,  one  of  the  salient  features 
of  infantile  paralysis,  and  seems  to  show  itself  more  quickly,  in 
this  disease,  than  in  cases  of  lésions  of  mixed  nerves.  wliere,  how- 
ever,  it  is  very  rapid.  Thus,  according  to  Dr.  Duchenne  (de  Bou- 
logne), it  is  already  very  évident  at  the  end  of  a  month,  in  infantile 
paralysis  ;  and  there  are  cases,  thougli  indeed  very  rare,  in  which 
ït  may  be  remarked  even  from  the  first  days. 

b.  Arrest  of  developmeni  of  the  osseous  System. — Wehaveto  note 
liere  an  important  feature  described  by  M.  Duchenne  (de  Boulogne), 
and  after  him,  by  Herr  Volkmann,  namely,  the  arrest  of  develop- 
ment  of  the  osseous  System.  The  atrophy  which  affects  the  bones 
is  not  at  ail  in  necessary  proportion  to  the  degree  or  extent  of  the 
muscular  paralysis  and  wasting. 

Thus,  according  to  a  remark  of  Duchenne  (de  Boulogne)  an  ex- 
tremity,  stricken  with  infantile  paralysis,  may  hâve  lost  most  of  its 
muscles,  and  yet  be  no  shorter  than  that  of  the  opposite  and  healthy 
side,  except  by  two  or  three  centimètres  ;  whilst,  in  another  case, 
the  loss  in  length  of  the  paralysed  extremity  may  reach  live  or  six 
centimètres,  although  hère  the  muscular  lésion  may  hâve  remained 
localised  in  barely  one  or  two  muscles  at  most,  and  may  hâve 
allowed  a  prompt  recovery  of  motion.^  H.  Yolkmann,  also,  lias 
observed  cases  of  considérable  shortening  of  the  affected  member  in 
children  who,  on  account  of  the  slight  degree  of  altération  in  the 
muscles  of  the  feet,  and  the  small  extent  of  essential  déformations, 
scarcely  halted  at  ail,  and  kept  on  their  legs  a  good  part  of  the  day. 
He  states  that  he  has  four  or  fire  times  noticed,  that  an  infantile 
paralysis  which  was  quite  transient,  and  which  issued  a  few  days  later 
in  complète  recovery  of  the  functions  of  the  muscles,  was  neverthe- 
less followed  by  trophic  osseous  lésions  which  lasted  for  life.^ 

It  would  be  difîicult  to  lind  an  example  better  adapted  to 
estabhsh  the  direct  action  of  lésions  of  the  central  nervous  system 

*  'De  l'electrisation  localisée/  3e  édition,  1872,  p.  400. 

^  R.  Volkmann,  loc.  cit.,  p.  6.  "Even  in  very  circumscribed  and  very 
incomplète  infantile  paralysis,  the  tropliic  disdrder  in  question  may  affect  the 
limb  tliroughout  its  whole  extent;  traces  are  frequently  foiuul  in  the  trunk, 
the  pelvis,  the  shoulders,  and  in  soine  cases,  even  in  the  head."     Id.  loc.  cit. 


COLDNESS.   DEFOEMATION.  133 

over  tlie  nutrition  of  the  osseous  parts,  since  it  is  impossible,  in 
such  circumstances,  to  appeal  to  tlie  influence  of  prolonged  func- 
tional  inertia. 

c.  Coldness  qf  the  exiremities. — Another  phenomenon,  which 
deserves  to  be  uoticed  on  the  same  grounds  as  the  preceding,  is  the 
often  very  marked  permanent  coldness  which,  sooner  or  later,  affects 
the  paralysed  extremity.  This  perhaps  is  the  place  to  point  out 
that,  besides  the  atrophy  of  the  muscles  and  the  bones,  we  find,  on 
autopsy,  in  cases  of  this  kind,  a  remarkable  diminution  in  the 
calibre  of  the  vascular  trunks.  There  are  circumstances  where  the 
coldness  in  question  becomes  appréciable  at  a  very  early  hour^ 
occasionally  some  weeks  after  the  invasion  or  even,  sooner.^ 

d.  A  final  feature  is  supplied  by  the  déformations  which  become 
évident  in  the  paralysed  member,  in  conséquence  of  the  prédominant 
action  of  those  muscles  which  hâve  remained  healthy  or  which,  at  a. 
given  moment,  hâve  recovered  tlieir  tone.  The  pathogeny  of  thèse 
déformations  présents  nothing  obscure.  We  know  that  the  atrophy 
is  not  uniformly  distributed  over  ail  the  muscles  of  a  limb  ;  it 
prédominâtes  in  certain  muscles  and  groups  of  muscles  ;  and 
the  antagonists  of  thèse  must,  in  the  long  run,  impose  vicious 
attitudes  according  to  the  direction  of  their  motion.  The  defor- 
mities  begin  to  show  themselves  about  the  eighth  or  tenth  month. 
Thus  is  developed  the  club-foot  of  infantile  paralysis  which  is  pre- 
eminently  the  paralytic  diih-foot,  and  which,  in  the  immense 
majority  of  cases,  assumes  the  form  of  varus  eqiàmis. 

There  is  extrême  laxness  of  the  ligaments,  and  it  is  easy  to  place 
the  parts  of  the  paralysed  limb  in  the  most  abnormal  attitudes, 
reminding  you  of  the  postures  of  a  puppet.  Taken  with  the  other 
characters,  and  especially  with  the  permanent  coldness  of  the  limb, 
this  extrême  laxity  of  the  joints  allows  us  to  distinguish,  with 
certainty,  the  club-foot  due  to  infantile  paralysis  from  congénital 
club-foot,  even  though  ail  information  touching  the  origin  of  the 
deformity  be  absent." 

From  the  epoch  when  the  lésions  hâve  become  definitely  esta- 
blished  in  certain  muscles,  it  may  be  said  that  the  disease  has 
become  arrested.  Henceforth,  we  hâve  only  to  deal  with  a  more 
or  less  distressing  infirmity   which,  according  to  the  remark  of 

^  Duchenne  (de  Boulogne)  mentions  that  he  has  observed  it  ah-eady  presenJ^ 
from  the  fourth  to  the  fifth  day.     Loc.  cit.,  p.  398. 
^  Heine,  loc.  cit.,  pp.  14,  15,  20. 


134  PATHOLOGICAL    ANATOMY. 

Heine,  appears  to  hâve  no  direct  influence  over  tlie  duration  of  life. 
In  support  of  this  statement,  I  can  introduce  to  your  notice  to-day 
an  aged  inliabitant  of  tliis  hospital  who  présents,  after  the  lapse  of 
threcscore  and  ten  years,  tlie  well-marked  characteristics  of  the 
disease  which  attacked  her,  when  she  was  five  years  old. 

Such  are  the  fundamental  characters  of  infantile  spinal  paralysis 
considered  in  its  régulai  course  ;  sometimes,  in  the  natural  évolu- 
tion of  the  disease,  irregularities  take  place  which  must  also  claim 
your  attention. 

Thus,  there  are  cases  in  which  the  initial  fever  exhibits  an  exeep- 
tional  intensity  and  continuance  ;  there  are  others  where,  subséquent 
to  the  fever,  the  paralysis  instead  of  suddenly  reaching  its  highest 
•degree  of  intensity,  develops,  on  the  contrary,  in  a  progressive 
manner  for  the  space  of  some  days,  or  even  of  some  weeks. 

Pinally,  there  are  other  cases  in  which,  during  the  period  of 
régression,  halting  stations  occur,  or  returns  of  active  aggression 
may  take  place.^ 

I  shall  not  insist  further  on  thèse  abnormal  facts  which,  besides, 
seem  to  be  rather  rare.  However,  I  did  not  think  they  should  be 
passed  over  in  silence  ;  because,  in  my  opinion,  they  may  serve  to 
establish  a  Connecting  link  between  infantile  spinal  paralysis  and 
the  other  diseases  of  this  group. 

III. 

I  shall,  at  présent,  endeavour  to  describe  to  you  the  lésions 
which  récent  researches  hâve  shown  to  exist  in  infantile  paralysis, 
and  to  which  the  very  remarkable  array  of  phenomena  just  men- 
tioned  belong. 

We  shall  treat,  in  the  first  place,  of  the  muscular  lésions  ;  and, 
in  the  second,  of  lésions  of  the  nervous  system. 

1°.  Lésions  ofthe  muscles. — I  will  be  brief  in  dealing  with  the 
question  of  muscular  change,  for  this  is  a  subject  that  still  requires 
further  examination. 

A.  First  per'iod.  —  Positive  data  concerning  the  histological 
altération  of  the  muscles  are  chiefly  lacking  in  connection  with  the 
early  phases  of  the  disease.  According  to  what  is  known,  the 
greater  part  of  the  primary,  or  ultimate,  fascicles  undergo  simple 
atrophy,  in  this  first  period,  without  fatty  degeneration.  Micro- 
scopical  examination,  in  fact,  shows  a  large  number  of  fascicles  of 
.'  See  Heine  and  Duchenne  (de  Boulogne)  fils,  loc.  cit.,  p.  8. 


LESIONS   OF  MUSCLE.  135 

very  small  diameter  which,  however^  hâve  preserved  their  normal 
striation,  and  which  exhibit  no  signs  of  fatty  degeneration.  A 
large  number  of  other  fascicles,  intermingled  with  thèse,  also 
enclose,  at  intervais,  clusters  of  nuclei  of  sarcolemma.  Finally, 
hère  and  there,  we  meet  with  a  third  order  of  fascicles,  generally 
very  few  in  number,  which  hâve  lost  their  striation  and  présent,  in 
différent  degrees  of  development,  the  cliaracters  of  fatty  granular 
degeneration.  This  is,  however,  I  repeat,  an  exceptional  occur- 
rence. In  short,  it  appears  established  that  irritative  lésions  pre- 
dominate  over  the  lésions  which  are  termed  passive.  We  shall 
soon  see  that,  contrary  to  the  opinion  generally  accepted,  the  same 
characteristic  is  observable  in  progressive  muscular  atrophy  of 
spinal  origin. 

The  lésions  in  question  appear  to  manifest  themselves  early  ;  M. 
Damaschino,  as  we  learn  from  M.  Duchenne  (de  Boulogne), 
reraarked  them  three  weeks  after  the  invasion  of  the  disease,  on  a 
fragment  of  muscle  obtained  by  the  assistance  of  the  "  emporte- 
pièce  ;"  with  the  same  instrument,  Drs.  Volkmann  and  Steudener 
were  likewise  able  to  study  the  paralysed  muscles,  at  a  period 
closely  following  the  outset,  and  they  liave  recognised  the  same 
altérations. 1  The  last-named  authors  point  eut,  also,  a  hyperplasia 
of  the  connective  tissue,  which  is  not  mentioned  by  the  other 
observers,  and  which  we  hâve  ourselves  identified  very  distinctly 
in  cases  of  old  standing. 

B.  Second  period.-^ïî  the  altered  muscles  be  examined  at  an 
epoch  remote  from  the  invasion  of  tlie  paralysis,  as  we  many  a  time 
hâve  had  occasion  to  do  at  the  Salpêtrière,  it  is  seen  that  ail  the 
signs  of  fatt^  substitution  and  loading  are  usually  superadded  to 
the  lésions  above  described.  Clusters  of  granulations  and  fatty 
droplets  accumulate  in  the  sheaths  of  the  sarcolemma,  and  replace 
hère  the  primary  (or  ultimate)  fascicle  which  totally  disappears,  or 
which  leaves  but  fragments  behind.  On  the  other  hand,  adipose  cells 
form  in  heaps  on  the  outside  of  the  sarcolemma,  in  the  intervais 
which  separate  the  primary  fascicles.^  This  interposed  adipose 
tissue  is  sometimes  sufficiently  abundant  to  distend  the  enclosing 
aponeuroses,  so    that,   as    M.    Laborde  ^  perfectly   observed,  the 

'  Volkmann,  loc.  cit.,  p.  5. 

^  See,  in  référence  to  this  subject,  in  the  second  volume  of  the  'Archives 
de  Physiologie,'  observations  of  MM.  Vulpian,  Charcot,  a^â  Joffroy. 
^  Laborde,  loc.  cit.,  p.  47. 


136  FATTY    SUBSTITUTION. 

volume  and  form  of  the  muscular  masses  may  be  preserved  to  a 
certain  extent,  thougli  most  of  the  ultimate  fibres  hâve  vanished. 
There  are  even  cases,  one  of  which  I  hâve  myself  observed/  where 
the  fatty  accumulation  is  so  great  in  extent  that  the  volume  of 
the  muscle  is  markedly  augmented,  so  as  to  exactly  reproduce  the 
appearance  seen  in  the  ultimate  period  of  the  affection  described 
by  Duchenne  (de  Boulogne)  under  the  name  ç,i  pseudo-hypertrophie 
or  mijo-sclerotic  paralysis.  This  is  a  point  concerning  which  you 
must  hâve  a  clear  understanding  ;  I  will  shortly  hâve  occasion  to 
show  you  that,  in  spite  of  this  analogy  of  secondary  importance, 
infantile  paralysis  still  differs  essentially  from  pseudo-hypertrophie 
paralysis  (the  atrophia  musculorum  lipomatosa  of  some  German 
authors)  by  an  imposing  array  of  clinical  and  necroscopic  cha- 
racters.  Let  it  suffice,  for  the  moment,  to  mention  that  the  spinal 
lésion  which,  in  infantile  paralysis,  is  never  absent,  is  absolutely 
déficient,  on  the  contrary,  in  myo-sclerotic  paralysis, — if  at  least  I 
may  judge  from  my  own  observations,  which  are  in  conformity  on 
this  point  with  those  of  Cohnheim. 

Pat  accumulation,  although  it  is  usual  in  infantile  amyotrophy 
of  long  standing,  is  not,  however,  necessarily  bound  up  with  it  ; 
side  by  side  with  muscles  distended  by  fat,  there  are  frequently  others 
which  are  reduced  to  a  very  small  volume,  in  which  the  adipose 
tissue  is  almost  entirely  déficient.^  In  the  latter  muscles,  we  only 
find  primary  fibres  of  very  small  diameter,  but  which  hâve  pre- 
served their  striation  ;  hère  and  there  some  sheaths  of  sarcolemma 
enclose  clusters  of  nuclei.  Thèse  wasted  primary  fibres  are  sepa- 
rate,  one  from  another,  by  fibrillary  connective  tissue,  which  is 
evidently  of  new  formation.  The  muscles  which  hâve  undergone 
this  mode  of  altération  présent,  to  the  naked  eye,  the  appearance  of 
fibrous  tissue,  or  of  dartos.  It  would  be  interesting  to  know  if 
the  interstitial  connective  hyperplasia  which  is  observed,  in  such 
cases,  is  a  constant  fact,  and  if  it  dates  back  to  the  first  phases  of 
the  disease,  as  the  observations  of  MM.  Yolkmann  and  Steudener 
would  lead  us  to  suppose.  But  this  is  a  point  which  requires 
further  investigation. 

2°,  Lésions  of  the -nervoiis  System  ;  spinal  lésions.  The  spinal 
lésions,  which  I  am  about  to  discuss,  unquestionably  constitute, 
at  the  présent  time,  at  once  the  most  interesting  and  ihe  newest 

1  'Arch.  de  Physiologie,'  t.  ii,  p,  142. 

'  Sec  the  observation  of  Wilson,  in  '  Arch.  de  Physiologie/  loc.  cit. 


SPINAL    LESIONS.  137" 

élément  in  the  anatomical  history  of  infantile  paralysis.  Ilence,  I 
think  it  will  be  useful  in  connection  witli  this,  to  enter  into  some 
détails. 

Many  authors^  as  you  are  aware,  hâve  considered  the  af- 
fection in  question  as  occupying  the  peripheral  parts,  muscles  or 
nerves  ;  others  hâve  looked  upon  it  as  an  essential  diseuse,  which, 
hère,  especially^  has  no  great  meaning.  It  is,  however,  right  to 
observe  that  the  majority  of  physicians  who  hâve  particularly  taken 
up  the  question  hâve,  with  common  consent,  designated  the  spinal 
cord  as  being  the  organ  where  the  primordial  and  fundamental 
lésions  of  infantile  paralysis  should  be  sought  for.  This  was  a 
correct  assumption,  on  their  part,  but  it  was  one  which,  until 
later  years,  was  not  based  upon  any  really  positive  data.  Kefer- 
ence  was  made  to  "contestions,''  to  " exudations ,"  without  any  strict 
démonstration  of  their  existence  ;  for,  in  the  absence  of  sufficient 
means  of  investigation,  the  results  of  necroscopic  examination 
were  nearly  always  négative  or  equivocal.  Such  was  the  state  of 
the  question  when,  at  the  Salpêtrière,  the  first  regular  studies 
were  undertaken  in  référence  to  the  necroscopy  of  the  spinal 
centre  in  infantile  paralysis. 

In  1864,  we  had  detected,  M.  V.  Cornil,  then  my  clinical  clerk, 
and  myself,  in  connection  with  a  case  observed  in  one  of  my  wards, 
a  portion  of  the  spinal  altérations  which  préside  over  the  develop- 
ment  of  infantile  paralysis.  But  it  was,  I  must  say,  the  least  im- 
portant portion.  Thus,  we  identified  the  existence  of  an  atrophy 
of  the  anterior  cornua  of  the  grey  substance,  and  of  the  antero- 
lateral  white  columns,  in  the  région  of  the  cord  whence  were 
given  off  the  nerves  going  to  supply  the  wasted  muscles  ;  but 
we  did  not  remark  the  decrease  in  number  and  in  volume  whick 
the  great  motor  cells  had  undergone, — an  altération,  however, 
which  may  be  very  distinctly  observed  in  a  section  prepared  at. 
that  period  by  M.  Cornil,  and  which  is  at  présent  in  tlie  possessioa 
of  my  friend,  M.  Duchenne  (de  Boulogne).^ 

The  lésion  of  the  motor  nerve-cells  in  infantile  paralysis  was 
first  pointed  ont  by  MM.  Vulpian  and  Prcvost,  in  1866,  in  the 
case  of  a  female  inmate  of  the  8alpôtrière.  In  this  case,  which, 
was  communicated  to  the  Société  de  Biologie  by  M.  Prévost,  most 
of  the  cells  had  disappcared  from  the  anterior  cornua  of  the  seg- 
ment of  the  cord  corresponding  to  the  atrophied  muscles  and,  in, 
*  '  Comptes  Rendus  de  la  Socictcde  Biologie,'  1864,  p.  187. 


138  niSTOEICAL    SKETOH. 

the  place  they  had  occupied,  the  neuroglia  exhibited  sclerous 
transformation.-^ 

A  case,  reported,  iu  1869,  by  MM.  L.  Clarke  and  Z.  Johnson, 
under  the  naine  of  nmscular  atrophy,  ought,  we  think,  to  be  cor- 
related  to  the  foregoing  :  a  critical  examination,  in  fact,  allows  us 
to  j)erceive  that,  though  thèse  authors  do  not  state  it,  the  case  in 
question  was  oue  of  infantile  paralysis.  The  period  of  life  in 
which  the  disease  showed  itself,  the  abruptness  of  the  invasion, 
the  mode  of  localisation  of  the  atrophy  in  the  muscles,  permit 
but  little  doubt  to  exist  in  this  respect  :  now,  in  this  instance  also, 
microscopic  examination  revealed  the  atrophy  of  the  anterior 
cornua,  the  disappearance  or  granular  wasting  of  a  certain  number 
of  motor  nerve-cells,  and  in  addition,  the  existence  of  several 
fociof  disintegratïon  in  différent  points  of  the  grey  substance.^ 

But,  if  I  mistake  not,  the  study  which  has  most  contributed  to 
détermine  the  character  of  the  spinal  lésions  of  infantile  paralysis, 
is  that  which  was  carried  out,  last  year,  by  M.  Joffroy,  my  clinical 
clerk,  and  myself,  in  a  very  remarkable  case,  that  of  a  female 
patient  of  my  wards,  named  Wilson,  who  succumbed  to  pul- 
monary  phthisis  at  the  âge  of  forty-five.  The  paralysis  had,  in 
lier  case,  suddenly  developed  itself,  when  she  was  seven  years  old; 
the  four  extremities  were  stricken,  and  most  of  their  muscles  had 
rapidly  atrophied.  Her  limbs  had,  also,  experienced  a  remark- 
able arrest  of  development,  and  presented  some  characteristic 
déformations.^ 

Hère  the  lésions  were  extremely  distinct,  and  they  extended 
nearly  the  whole  height  of  the  spinal  cord  :  throughout,  they 
occupied  principally,  and  in  some  parts  exclusively,  the  anterior 
cornua  of  the  grey  substance  (î^ig.  8.) 

In  ail  the  régions  of  the  cord  the  great  motor  cells  were  greatly 
altered,  though  in  différent  degrees;  and  in  the  most  seriously 
affected  parts,  entire  groups  of  cells  had  disappeared  without 
leaving  a  trace  behind.  The  neuroglia  had  almost  always  under- 
gone  sclerous  transformation  in  the  immédiate  neighbourhood,  and 
to  a  certain  distance  from  the  injured  cells,  but  there  were  points, 
and  this  fact  deserves  prominence,  where  this  cell-lesion  was  the 
only  altération  which  histologie  examination  could  detect,  the  con- 

^  Idem,  1866,  p.  215. 

=*  'Med.-Cliir.  Tnins.,'  t.  li,  London,  1868. 

^  '  Socicté  de  Biologie/  and  'Archives  de  Pliysiologie,'  t.  iii,  p.  135,  1870. 


LESIONS  OF  MOTOR  CELLS. 


139 


nective  web  having,  in  thèse  places,  retained  its  transparency,  and 
very  nearly  ail  the  cliaracteristics  of  its  normal  structure. 


EiG,  9. — Section  of  tlie  cord,  in  the  cervical  région,  in  a  case  of  infantile 
spinal  paralysis  of  the  right  superior  extremitj.  Préparation  obtained  in 
the  Sali)êtrière,  in  the  case  of  a  female  patient  who  died  of  gênerai  paralysis 
at  the  âge  of  fifty.  Fibroid  atrophy  of  the  anterior  cornu  of  the  right  side, 
consécutive  eniaciation  of  ail  the  white  fascicles  in  the  corresponding  half 
of  the  cord. 

Finally,  we  shall  notice,  in  our  observations,  an  atropby  with 
partial  sclerosis  of  the  antero-lateral  columns  and  a  well-marked 
wasting  of  the  anterior  roots,  particularly  remarkable  on  a  level 
with  the  régions  of  the  cord  which  were  inost  gravely  affected, 
altérations  which  had  been  already  pointed  out  in  essays  published 
anterior  to  our  own. 

In  the  memoir,  based  upon  our  observations,  we  considered  our- 
selves  justified  in  admitting  that  the  lésion  of  the  molor  nerve-cells, 
mentioned  already  in  the  cases  of  MM.  Yulpian  and  Prévost,  and  in 
that  of  L.  Clarke,  is  a  constant  fact  in  infantile  spinal  paralysis, 
and  one  front  loJnch  the  principal  symptoms  of  the  disease  are 
derived,  particularly  the  paralysis  itself,  and  the  muscular  atrophy 
as  well.  We  also  gave  it  as  our  opinion  that,  in  ail  probability, 
that  is  the  initial  anatomical  fact  ;  the  lésions  of  the  neuroglia  and 
the  atrophy  of  the  nerve-roots  being  regarded  as  consécutive 
phenomena. 


140  SECONDAEY  ALTERATIONS. 

I  cannot,  to-daj,  give  you  ail  the  arguments  wliicli  raight  be 
pleaded  in  support  of  thèse  assertions;  to  do  so,  would  be  to 
occupy  too  mucli  time.  Besides,  I  reserve  this  task  for  the  period 
when  I  shall  hâve  made  known  the  other  morbid  species  which 
belong  to  the  group  of  myopathies  of  spinal  origin.  I  expect 
then  to  enter  upon  a  regular  discussion  of  the  subject,  in  référ- 
ence to  the  part  which  I  attribute  to  the  motor  nerve-cells  in  the 
production  of  trophic  lésions  of  the  muscles.  At  présent,  I 
shall  confine  myself  to  the  following  considérations  which  more 
particularly  concern  infantile  paralysis. 

In  connection  with  our  first  conclusion,  it  will  be  enough  to 
point  ont  that  it  receives  confirmation  from  ail  the  facts,  and  they 
are  now  numerous  enough,  which  hâve  been  observed  since  the 
publication  of  our  meraoir. 

Thus,  the  lésion  of  the  motor  cells  is  expressly  mentioned  in  an 
observation  reported  by  MM.  Parrot  and  Jofi'roy,  the  case  being 
that  of  a  child  where  the  disease  had  barely  lasted  a  year  ;  Mn  a 
case  noted  by  M.  Vulpian  at  the  Salpêtrière  ;  ^  and  in  two  other 
cases  observed  at  the  Children's  Hospital,  by  M.  Damaschino,  the 
détails  of  which  I  only  know  as  yet  from  the  information  given 
by  M.  Duchenne  (de  Boulogne).^  Finally,  the  self-same  lésion 
existed,  in  the  most  distinct  manner,  in  three  new  cases  quite 
recently  noted  in  my  wards,  the  anatomical  examination  of  which 
has  been  carried  on  with  the  greatest  care  by  my  pupils,  MM. 
Michaud  and  Pierret.  Thèse  new  facts,  taken  in  conjunction  with 
the  préviens  ones,  go  to  form  a  sufficiently  imposing  array, 
especially  when  it  is  remembered  that,  up  to  the  présent  time,  no 
contradictory  case  of  any  value  has  been  reported.  The  cases 
which  hâve  been  quoted  in  opposition  to  us  ail  date  from  a  period 
when  the  raethods  of  investigation  as  applied  to  the  anatomical 
study  of  the  cord  had  not  reached  the  degree  of  perfection  which 
they  possess  at  présent;  and,  besides,  none  of  thèse  cases  bears 
that  character  of  exactness  which  we  hâve  a  right  now  to  require 
in  observations  of  this  kind. 

^  '  Archives  de  Physiologie,'  t.  iii,  1870. 

2  Idem,  t.  iii,  1870. 

3  The  observations,  three  in  number,  noted  in  the  wards  of  M.  Roger  by 
M.  Damaschino,  hâve  been  recently  communicated  to  the  Société  de  Biologie, 
and  publislied  in  extem-o  in  the  'Gazette  Médicale,'  Nos,  41,  43,  45,  48,  51 
(October,  November,  December,  1871). 


SECONDAEY   ALTERATIONS. 


141 


With  respect  to  the  second  proposition^  I  shall  put  forward  what 
foUows  : — If,  in  certain  points,  the  lésions  of  the  neuroglia  invade 
the  greater  portion  of  the  grey  substance,  and  even  sometimes  ex- 
tend  to  the  adjacent  parts  of  the  antero-lateral  columns,  it  is  not  the 
less  true  that,  in  other  points,  they  remain  strictly  limited  to  the 
anterior  cornua,  which  they  do  not  always  even  occupy  throughout 
their  whole  extent.  They  are,  in  fact,  occasionally  seen  strictly,  and  as 
it  were  systematically,  localised  in  the  very  circumscribed  oval 
space  which  corresponds  to  a  group  or  cluster  of  motor  cells. 
(fig-  9-) 


FiG.  10.— Section  of  the  cord,  in  the  lumbar  région,  a,  left  auterior  cornu, 
healthy  ;  «,  liealthy  ganglionie  nucleus.  B,  right  anterior  cornu  ;  b,  médian 
ganglionic  nucleus,  the  cells  of  which  are  destroyed,  and  whicli  is  repre- 
sented  by  a  little  focus  of  sclerosis. 

How  is  it  conceivable  that  this  could  be,  if  the  altération  had 
its  starting-point  in  the  connective  tissue  interposed  between  the 
nerve-elements  ?  Is  it  not  more  probable  that  it  has  its  origin  in 
spécial  organs  endowed  with  proper  functions,  such  as  the  great 
nerve-cells,  termed  motor,  are  ?     It  is  in  this  way,  according  to 


142  LESIONS    OP    THE   NEUROGLIA. 

the  theory  put  forth  by  M.  Vulpian,  a  theory  to  which  I  quite 
adhère,  that  the  scléroses  systematically  limited  to  the  posterior 
columns  should  be  correlated  to  an  irritation  primarily  occupying 
the  nerve-tubes  which  enter  into  the  composition  of  thèse  columns. 

There  are  occasions,  also,  and  the  case  of  Wilson  may  be  hère 
remembered,  where  in  certain  points  the  altération  of  a  certain 
number,  it  may  be  an  entire  gronp  of  nerve-cells,  is  the  only  lésion 
which  histological  examination  enables  us  to  perceive,  the  con- 
nective  web  having,  in  such  points,  preserved  its  transparency,  and 
very  nearly  ail  the  characteristics  of  normal  structure.  In  other 
régions,  the  lésions  of  the  neuroglia  may  show  themselves  much 
more  marked  towards  the  central  part  of  an  aggregation  of  nerve- 
cells  than  in  the  peripheral  portion  ;  much  more  marked  likewise 
in  the  immédiate  vicinity  of  the  cells  than  in  the  intervais  between 
them,  so  that  the  cells  shall  appear  as  so  many  centres  or  foci 
whence  the  morbid  process  has  radiated,  to  a  certain  distance,  in  ail 
directions. 

Again,  it  could  not  be  admitted  that  the  irritation  had  originally 
developed  in  the  peripheral  parts,  and  that  it  had  afterwards 
ascended  to  the  central  portion  by  the  channel  of  the  anterior 
roots  of  the  spinal  nerves  ;  for  the  latter  generally,  as  MM.  Parrot 
and  Joffroy  hâve  clearly  shown,  only  présent,  in  récent  cases,  at  a 
level  with  the  altered  régions  of  the  cord,  comparatively  slight 
lésions,  and  not  at  ail  proportionate  in  intensity  to  the  lésions  of 
the  grey  substance. 

It  appears  évident  to  us,  frora  what  précèdes,  that  the  motor 
nerve-cells  are,  in  ail  reality,  the  primary  seat  of  the  disease. 
Generally,  no  doubt,  the  irritative  process  subsequeutly  attains 
the  neuroglia,  and  extends,  bit  by  bit,  to  the  différent  régions  of 
the  anterior  cornua  ;  but  that  is  not  at  ail  necessary.  A  fortiori, 
we  must  regard,  as  a  consécutive  and  purely  accessory  fact,  the 
extension  observed,  in  certain  cases,  of  the  morbid  process  to  the 
antero-lateral  columns. 

The  lésion,  in  question,  of  the  nerve-cells,  to  judge  from  the  cha- 
racter  of  the  altérations  presented  by  the  connective  web,  is  of  an 
irritative  nature;  but,  this  is  a  point  as  to  which  direct  and 
purely  anatomical  examination  cannot,  at  least  at  présent,  afford  us 
any  information.  Similarly,  in  fact,  to  what  takes  place  with 
respect  to  the  nerve-tubes,  the  irritated  nerve-cells  atrophy  and, 
at  the  latter  end  of  the  process,  disappear,  without  the  mode  of 


FIBEOID    TRANSFORMATION.  143 

the  affection  which  originates  them  being  revealed  by  any  spécial 
characters. 

Oiie  Word,  in  concluding,  relative  to  thèse  changes  in  the  connec- 
tive  web  which,  in  mj  opinion,  are  a  secondary  phenomenon,  con- 
sécutive on  the  afifection  of  the  nerve-cells.  In  cases  of  old 
standing,  they  principally  consist  in  a  fibrillary  or  fibroid  nieta- 
morphosis  of  the  reticulum,  with  more  or  less  complète  disappear- 
ance  of  nerve-tubes  and  condensation  of  the  tissue  ;  but  thèse  are 
only  the  last  vestiges  of  a  morbid  process  which  bas  been  long 
extinct,  and  it  is  difficult  to  divine  what  may  be  the  altérations  in 
the  early  phases.  It  is  probable  enough  that  we  might  there  fiud 
the  histological  characters  of  acute  myelitis  with  multiplication  of 
myelocytes  and  of  the  nuclei  of  the  vascular  sheaths,  such,  in  short, 
as  it  bas  been  described  by  Frommann  and  by  Mannkopf.  The 
existence  of  foci  of  disintegration,  noticed  in  Clarke's  observation, 
and  in  some  of  the  cases  we  hâve  noticed  at  the  Salpctrière,  shows 
that,  in  some  spots,  the  inflamed  tissue  may  undergo  real  dissociation. 
The  cases  of  M.  Damaschino  would  even  go  to  establish  that,  in  the 
most  seriously  injured  points  of  the  cord,  we  may  meet  with  ail  the 
characters  of  destructive  myelith  with  formation  of  a  focus  of  red 
softening  with  vascular  lésions,  granular  bodies,  and  the  rest  of  it. 
However  this  may  be,  you  will  easily  understaud,  gentlemen,  that 
nothing  of  ail  this  goes  to  invalidate  the  theory  according  to  which 
the  apparatus  of  motor  nerve-cells  is  the  first  focus  and  starting- 
point,  as  it  were,  of  the  inflammatory  process. 

It  remains  for  me  now  to  set  the  symptoms  face  to  face  with  the 
lésions,  and  to  inquire  how  the  former  are  derived  frora  the  latter, 
a  duty  which  I  shall  shortly  endeavour  to  perform. 


LECTURE  X. 

SPINAL  PARALYSIS  OP  THE  ADULT.  '^EW  RESEAECHES 
CONCERNING  THE  PATHOLOGICAL  ANATOMY  OF  IN- 
PANTILE  SPINAL  PARALYSIS.  AMYOTROPHIES  CONSE- 
CUTIVE ON  ACUTE  DIFFUSE  SPINAL  LESIONS. 

SuMMARY. — Spinal  paralyds  of  tlie  adult.  History.  De- 
scription of  a  case,  horrowedfrom  M.  Buchenne  [de  Boulogne) . 
Personal facts.  Close  analogies  Connecting  acute  sj)inal para- 
l'i/sis  of  tJie  adult  with  tliat  of  tlie  cliild.  Symptomatological 
modifications  due  ta  âge.     Prognosis. 

Récent  îvorJcs  concerning  tlie  pathological  anatomy  and 
physiology  of  infantile  spinal  paralysis  ;  they  confrm  in 
essential points,  and  complète  in  certain  respects,  tlie  results 
already  detailed. 

A  Word  as  to  acute  spinal  lésions  which  are  not,  as  in  infan- 
tile 2)aTalys'is,  systematically  limited  to  tlie  anterior  cornua  of 
the  grey  substance.  Acute  central  gêner alised  myelitis,  hœma- 
tomyélia,  traumatic  myélites,  acute  partial  myélites.  Condi- 
tions in  wîdch  thèse  affections  détermine  the  rapid  atrophy  of 
the  muscles. 

I- 

Gentlemen, — It  is  now  a  long  time  since  M.  Duclieime  (de 
Boulogne)  noted  the  existence,  in  the  adult,  of  an  ac7ite  spinal  para- 
lysis, comparable  to  that  of  the  child.^  Dr.  Moritz  Meyer,^  of  Berlin, 
and  Dr.  Roberts,^  hâve  also,  in  past  days,  reported  cases  which 
evidently  belong  to  this  category.  I  hâve,  myself,  been  more  than 
once  struck  with  the  remarkable   resemblance  which   produces   a 

'  See,  in  référence  to  this  subject,  tlie  tbesis  of  M.  Duclienne  (de  Boulogne) 
fils. 

*  M.  Mejer,  'Die  Electricitât  und  ihre  Anwendung/  Berlin,  1868,  p.  aïo. 
^  Reynolds'  '  System  of  Medicine,'  t.  i,  p.  169. 


SPINAL    PARALTSIS    OF    THE    ADULT.  14-5 

clinical  connexion  between  certain  paraplegias,  of  abrupt  invasion 
followed  by  muscular  atrophy,  developed  in  youtli  or  in  adult  âge, 
and  the  paralysis  of  young  children. 

I  désire  to  establish  before  you  the  reality  of  tbe  existence  of  this 
spinal  paralysis  of  the  adult  comparable  to  infantile  spinal  paralysis. 
I  hope  to  succeed,  by  first  detailing  the  principal  features  of  an 
observation  which  I  borrow  from  the  new  édition  of  the  '  Traite 
d'Electrothérapie  '  by  Dr.  Duchenne  (de  Boulogne),  and  by  after- 
wards  describing  some  of  the  facts  which  hâve  corne  under  my 
Personal  knowledge. 

The  case  of  Dr.  Duchenne  (de  Boulogne)  relates  to  a  girl,  aged 
22,  who  awoke  one  morniug  with  fever,  pains,  and  dif&culty  in 
moving  her  limbs.  An  hour  later  she  complained  of  pains  in  the 
posterior  cervical  région,  formications  and  painful  irradiations  in 
her  fingers.  The  latter  circumstance,  if  you  refer  to  the  descrip- 
tion of  infantile  paralysis,  may  seem  to  constitute  a  striking  anomaly 
as  regards  the  matter  in  question  ;  but  we  hâve  not  failed  to  point 
ont  to  you,  elsewhere,  that  children,^  when  attacked  with  spinal 
paralysis,  often  complain  of  similar  pains,  when  they  are  old  enough 
to  express  their  feelings. 

Différence  of  âge,  however,  even  supposiug  a  fundamentally 
identical  process,  should  necessarily  cause  différences  which  must 
not  be  overlooked.  Thus,  for  instance,  in  cases  similar  to  that  which 
we  hâve  quoted  from  Dr.  Duchenne,  when  the  development  of  the 
patient  is  complète  at  the  period  when  the  disease  makes  its 
appearance,  you  could  not  expect  to  see  that  atrophy  by  arrest  of 
development,  which,  in  children,  goes  at  least  a  good  way  to  déter- 
mine the  shortening  of  the  affected  members,  and  forms  one  of  the 
most  salient  features  of  infantile  paralysis, 

Eeferring  again  to  the  case  of  Dr.  Duchenne  we  find  that  hardly  had 
the  pain  shown  itself  than  the  four  extremities  were  completely  para- 
lysed,  rendered  absolutely  inert.  Pour  days  later,  the  fever  had  ceased. 

The  paralysis  of  movement  persisted  for  two  months  without 
any  noticeable  change;  it  seemed,  I  repeat,  to  hâve  been  absolute; 
and,  in  spite  of  that,  the  sensibility  of  the  skin  was  not  at  ail 
affected.  Nor  was  any  lasting  disorder  of  micturition  ever  observed, 
nor  the  least  sign  perceived  of  the  formation  of  eschars. 

Towards  the  end  of  the  third  month,  the  retrogression  of  the 
paralytic  symptoms  began  to  become  manifest. 
^  See  Lecture  IX,  p.  130. 

VOL.   II.  10 


146  DK.    DUCHENNE's   case. 

At  first,  motion  was  gradually  regained  in  tlie  lower  extremities  ; 
tlien,  fifteen  days  later,  it  reapj)eared  in  tlie  upper  extremities,  but, 
iudeed,  in  an  imperfect  fasliiou.  The  fact  was  that  in  a  considér- 
able number  of  the  muscles  of  the  superior  extremities,  nutrition 
had  sufFered  to  sucb  an  extent  that  atrophy  was  already  visible. 

Six  months  after  tlie  invasion  of  the  paralysis,  an  attentive  ex- 
amination  revealed  the  existence  of  irréparable  disorders.  A  large  por- 
tion pf  the  muscles  of  the  arm,  fore-arm,  andhand  was  considerably 
wasted,  especially  in  the  right  liinb  ;  and,  in  addition,  they  gave  no 
reaction  under  the  stimulus  of  faradisation.  Contrary  to  what 
took  place  in  the  upper  extremities,  graduai  amendment  continued 
in  the  lower  limbs  ;  where  ail  the  muscles  had  recovered  their 
f  unctions,  with  the  exception  of  the  tibialis  anticus  of  the  right  leg, 
whose  altération,  and  the  conséquent  prédominant  action  of  the  anta- 
gonistic  muscles,  had  occasioued  a  sort  of  paralytic  talipes  equinus. 

It  is  scarcely  doubtful,  gentlemen,  that  in  spite  of  the  very  peculiar 
characters  of  the  symptomatic  group,  cases  of  this  kind  hâve  been 
oft  times  mistaken  or  misunderstood.  Now,  according  to  what  I 
hâve  read  or  seen,  the  form  of  spinal  paralysis  in  question  is 
far  from  being  absolutely  rare  among  the  diseases  of  adult  life.^ 

^  Several  cxamples  of  aduU  spinal  luiralysis  hâve,  iu  Ibe  last  few  years, 
beeu  reported  by  M.  Berûliardt  ('Archivfiu-  Pliysiatrie,' iv  Ed.,  1873)  aud 
Kussmaul  (Frey — Aus  der  medicinischeu  Klinik  der  Herru  Professer  Kuss- 
maul  in  'Berlin  Klin.  Wochenscli.,'  1874,  Nos.  i,  2,  and  3).  One  of  the 
cases  of  Professor  Kussmaul  is  particularly  interesting,  inasmuch  as  the  oscil- 
lations of  the  central  température  hâve  been  noted  during  the  whole  time  of 
the  initial  fébrile  period.  Thèse  cases  hâve  been  published  in  extenso  in  'Le 
Progrès  Médical,'  1874,  Nos.  ir  and  12.  In  Englaiid,  in  the  neighbourhood 
of  Leeds,  a  few  years  ago,  I  met,  whilst  with  my  friend  Professor  Browu- 
Séquard,  a  gentleman  aged  38,  wlio,  two  years  before,  after  being  out  of  sorts 
for  four  days,  was  taken  with  intense  fever  which  lasted  nearly  a  week,  aud 
was  foUowL'd  by  complète  motor  paralysis  of  the  four  extremities  with  abrupt 
development.  A  month  after  the  invasion,  motion  began  to  reappear  in  the 
right  arm,  first,  theu  gradually  in  the  other  members.  But  the  patient,  at 
présent,  exhibits  a  probably  indelible  atrophy,  particularly  évident  in  the 
muscles  of  the  right  arm  and  Icft  leg.  Excepting  some  formicatiou  no  sensory 
disturbance  lias  ever  been  experienccd.  The  functions  of  bladder  and  rectum 
bave  always  remained  normal.     No  bedsores  were  formed. 

A  case  published  by  Professor  Cuming  (of  Belfast)  in  the  '  Dublin  Quar. 
terly  Journal  of  Médical  Science,'  May,  1869,  p.  471,  seems  to  me  to  bclong, 
like  the  preceding,  to  adult  spinal  juirali/sis.  See,  in  référence  to  this  subject, 
the  interesting  work  of  a  student  of  the  Salpêtrière,  M.  Petitfils,  eutitled, 
'  Atrophie  aiguë  des  cellules  nerveuses.' 


PERSONAL    OBSERVATIONS.  147 

Still,  the  prognosis,  as  well  as  ail  the  otlier  circumstances  of  tlie 
disease,  generally  differs  liere  in  a  singular  way  from  wliat  is 
seen  in  the  other  forms  of  paraplegia  of  abrupt  invasion.  This 
is  a  fact  with  wliich  it  is  important  to  be  familiar.  Hence,  we 
do  not  liesitate  to  enter,  now,  into  new  détails,  in  référence  to 
two  cases  which  I  bave  receutly  noted,  and  which  are  full  of  signi- 
ficance  in  my  opinion. 

On  account  of  the  âge  of  the  subjects  concerned  (one  being  nine- 
teen,  the  other  fifteen  and  a  balf)  they  establish  a  sort  of  transition 
between  the  observation  which  précèdes  and  those  whicli  belong 
to  infantile  paralysis,  properly  so  called. 

Case  i. — M.  X — ,  aged  19.  The  only  particulars  anterior  to  the 
paralysis  which  deserve  notice  are  the  following  : — His  mother  was 
pregnant  thrice,  and  during  two  of  her  pregnancies  she  was  subject 
to  mental  disorder. 

The  health  of  X —  bad  always  been  excellent  ;  he  enjoyed  great 
physical  strength,  and  was  of  a  rather  placid  character.  During 
the  months  of  June,  July,  and  until  the  loth  of  August,  1873, 
X —  made  great  intellectual  efforts  in  preparing  for  an  examination. 
During  this  time  he  repeatedly  suffered  from  copions  epistaxis  to 
which  he  had  not  been  accustomed.  He  failed  in  his  examina- 
tion, and  suffered  great  vexation  in  conséquence. 

Such  were  the  circumstances  wlien,  on  the  1 6th  of  August,  X — 
was  observed  in  the  park  of  the  Château  where  he  lived,  breaking 
down  a  tree  with  feverish  activity.  Questioned  as  to  the  reason  of  his 
act,  he  replied — '^Iwant  to  break  something,  for  I  feel  myself 
irritated.^^  The  same  day  he  complained  of  great  fatigue,  a  feehng 
of  contusion  and  extrême  lassitude  {courbature) ,  especially  in  the 
lumbar  région,  and  suffered  from  copions  perspirations. 

Next  day,  he  felt  himself  worse.  He  was  able  to  get  up,  bow- 
ever,  but  he  could  only  walk  by  leaning  on  a  staff  or  on  the  arm  of 
his  serving  man. 

On  the  third  day,  a  feverish  state  set  in,  with  some  violence 
from  the  outset,  and  which  was  soon  accompanied  by  symptoms 
such  as  would  lead  one  to  believe  it  the  begiuning  of  an  attack  of 
typhoid  fever  supervening,  with  characters  of  great  gravity.  The 
tongue  was  dry  and  loaded  with  blackish  fur  ;  there  was  great  thirst  ; 
the  skin  was  bot;  the  puise  120;  there  was  delirium  during  thenight. 
rinally,  tympanitis  was  observed,  and  to  empty  the  bladder  it  was 
requisite  to  pass  the  cathéter  several  times  in  a  period  of  thirty-six 


148  PERSONy\L    OBSERVATIONS. 

liours.  It  is  important  to  note  tliat  tlie  rétention  of  urine  was,  as 
may  be  remarked,  quite  transitory.     It  dicl  uot  subsequently  recur. 

This  fébrile  ])eriod  terminated  at  the  end  of  five  or  six  days,  and 
the  gênerai  condition  of  the  patient  rapidly  became  quite  normal 
again.  Then  only  was  the  existence  of  an  almost  complète  para- 
lysis  of  motion  discerned,  which  was  marked  by  an  absolute  flaccid- 
ness  of  the  parts,  and  which  affected  equally  the  four  extremities. 
The  motor  inertia  had  been  already  remarked  during  the  course  of  the 
fever  ;  but  it  had  been,  until  then,  regarded  as  the  resuit  of  extrême 
adynamia.  There  never  was  any  tendency  to  the  formation  of  eschars. 

Things  remained  in  this  state  during  the  following  fortnight. 
After  the  lapse  of  this  time,  a  certain  amendment  was  experienced  in 
the  condition  of  the  upper  extremities  ;  and,  in  addition,  the  patient 
began  to  be  more  or  less  able  to  sit  up. 

I  was  called  to  see  M.  X — ,  for  the  first  time,  on  Nov.  i,  1873,. 
two  months  and  a  half  after  the  invasion  of  the  disease.  I  noted 
then  the  following  facts  : — On  both  sides,  but  especially  on  the 
right,  there  was  rather  well-marked  atrophy  of  the  shoulders  and  of 
the  posterior  part  of  the  arm  ;  on  the  contrary,  the  muscles  of  the 
fore-arms,  of  the  chest,  of  the  abdomen,  and  especially  of  the  neck, 
contrasted  by  their  prominence,  which  recalled  their  normal  state. 
On  both  sides,  but  chiefly  on  the  left,  the  palm  of  the  hand  was 
flattened  and,  as  it  were,  excavated,  in  conséquence  of  the  atrophy 
af  the  thenar  and  hypothenar  eminences.  Trom  time  to  time,  in 
the  muscles  of  the  hand,  there  appeared  spontaneous  fibrillary  con- 
tractions, which  caused  slight  movements  in  the  fingers.  X — 
cannot  raise  his  shoulders,  lift  his  arms,  nor  extend  his  fore-arm; 
but,  by  leaning  on  his  elbows,  he  can  use  his  hands  to  feed  himself. 
The  several  kinds  of  cutaneous  sensibility  are  in  no  degree  changed 
in  the  différent  portions  of  the  trunk  and  upper  extremities. 

As  to  the  inferior  extremities,  they  are,  both,  fiaccid,  inert, 
emaciated.  No  trace  of  contracture  or  retraction  is  observed.  The 
wasting  is  greater  in  the  tliighs  than  in  the  calves.  Yoluntary 
movements  are  almost  impossible  ;  on  the  left,  they  are  limited  to 
some  slight  motions  of  the  great  toe  ;  on  the  right,  ail  the  toes  may 
be  either  voluntarily  flexed  or  extended,  but  within  very  restricted 
limits.  It  is  carefully  noted  that  hère  also  cutaneous  sensibiKty  is 
not  the  least  changed  ;  it  is  also  particularly  noted  that  the  différent 
modes  of  stimulating  the  skin  provoke  no  refiex  movements. 

The  patient  expériences  no  distressing  sensation  in  the  paralysed 


PERSONAL    OBSERVATIONS.  149 

limbs.  He  says  that  lie  only  feels  some  formications  from  time  to 
time;  he  also  complains  of  a  fréquent  need  of  changing  position, 
more  pressing  at  night  than  by  day.  The  lower  extremities  are 
usually  cold^  chiefly  the  left  foot  and  leg^  which,  in  addition,  are 
usually  covered  with  clammy  sweat. 

The  puise  is  normal,  the  appetite  excellent,  the  sleep  only  broken 
by  the  need  of  changing  position,  just  described.  Thefunctions  of 
the  sphincters  are  regular. 

It  bas  been  found  impossible  to  ascertain  exactly  the  epoch  when 
the  atrophy  of  the  muscles  began  to  occur.  Assurance  was  given, 
however,  that  it  was  remarked  a  few  weeks  merely  after  the  inva- 
sion of  the  disease.  It  is  also  to  be  regretted  that,  for  want  of 
suitable  appliances,  an  electric  exploration  of  the  wasted  parts  could 
not  be  performed  at  this  period. 

In  the  consultation  which  took  place,  at  the  first  interview,  I 
essayed  particularly  to  emphasise  the  abrupt  and  almost  sudden 
invasion  of  the  paralytic  accidents,  marked  as  it  had  been  by  a 
very  distinct  fébrile  period, — the  flaccidness,  and  the  great  atrophy 
rapidly  supervening,  which  the  muscles  of  the  paralysed  limbs 
exhibited — phenomena  which  contrasted  with  the  absence  of  anses- 
thesia,  of  lasting  disorders  of  bladder  or  rectum,  and  of  sacral  eschars. 
I  stated  my  opinion  that  this  array  of  positive  or  négative  sym- 
ptoms  aUowed  us  to  approximate  the  case  of  M.  X^-,  to  the  type 
of  ivfantïle  spinal  par aly sis. 

Lastly,  basing  my  opinion  on  what  is  taught  by  the  natural 
history  of  this  affection,  I  considered  that  I  could  assert  that  the 
rétrocession  of  symptoms  which  had  already  begun,  in  the  upper  ex- 
tremities, would  become  still  more  manifest  there,  and  would  doubt- 
less  extend,  to  a  certain  degree,  to  the  lower  extremities  ;  that  it 
miglit  even  happen  that  it  would  again  be  possible  for  the  patient 
to  stand  and  to  walk  with  the  assistance  of  a  proper  apparatus  j 
that,  finally,  a  return  of  aggressive  symptoms  was  scarcely  to  be 
apprehended.^ 

The  subséquent  history  of  the  case  shows  that  thèse  prévisions 
■were  fulfiUed.  A  note,  taken  in  February,  1874,  states,  in  fact, 
that  a  vexy  remarkable  improvement  had  taken  place  as  regards  the 

^  I  am  informed  by  my  coUeague,  Dr.  Bouvier,  who  bas  had  great  expérience 
in  this  matter,  that  only  thrice  in  the  course  of  liis  long  career  has  he  seen 
-the  normal  rétrocession  of  the  symptoms  of  infantile  spinal  paralysis  impeded 
'by  a  relapse. 


150  PEKSONAL    OBSERVATIONS. 

power  of  motion,  and  nutrition  of  the  upper  extremities  ;  with 
respect  to  the  inferior  extremities,  faradaic  contractility  begins  ta 
reappear  in  several  muscles  wlicre  it  had  been  either  greatly  weak- 
ened,  or  abolislied.  On  the  other  hand,  on  account  of  the  pré- 
dominant action  of  the  posterior  muscles  of  the  thigh  and  those  of 
the  calves,  a  tendency  to  flexion  of  the  legs  has  occurred,  and  to 
the  formation  of  equinus  club-feet,  against  which  différent  appli- 
ances  hâve  been  tried. 

In  April,  muscular  strength  had  made  such  progress,  in  the 
lower  extremities^  that  the  patient  was  able  to  stand  and  waik  a  few 
steps  in  his  room,  with  the  help  of  two  assistants. 

Ëinally,  in  August,  about  a  year  after  the  invasion,  he  can,  when 
sitting,  rise  up  without  assistance  ;  and,  with  the  help  of  a  pair  of 
crutches,  he  takes  short  walks.  He  can  even  walk  a  little,  leaning 
on  a  cane  only,  by  using  an  apparatus  which  hinders  flexion  of  the 
left  knee.^ 

The  following  case,  altliough  less  regular  in  some  respects  than 
the  foregoing,  deserves,  however,  to  be  compared  with  it.  It 
may  be  approximated  to  those  temjporary  paralyses,  desuribed  by 
Kennedy,  the  history  of  which  cannot  be  severed  from  that  of 
permanent  infantile  pa ralysis. 

Case  3. — Charles  E — ,  at  présent  aged  fiftœn  years  and  a  half, 
is  a  tall,  well-made  youth,  of  an  inteJljgeait  appearance.  In  his 
antécédents,  no  disease  worth  notice  is  found;  no  convulsions. 
He  has  neither  experienced  any  severe  moral  émotion,  nor  received  a 
chill.  We  are  only  told  that  he  has  grown  much,  in  a  very  ahort 
space  of  time. 

On  the  ajth  September,  1873,11e  was  taken  with  a  fever  of  slight 
intensity,  which  did  not  oblige  him  to  take  to  his  bcd.  His  appe- 
tite,  however,  was  gone;  the  tougue  was  loaded.     The  fébrile  state 

1  During  tbe  period,  exteiiding  from  the  iQth  of  August,  1873,  to  January 
ist,  1874,  the  treatmeut  consktod  principally  in  the  application  of  cuppiiig 
with  scarification,  of  blisters,  and  then  of  the  actual  cautery  along  the  verté- 
bral column.  From  the  last-mentioned  date,  the  paralysod  or  wasted  muscles 
■were,  every  second  day,  subjected  to  tJie  stimulation  of  a  faradaic  ourrent  of 
médium  intensity.  In  addition,  X —  took  stryehninc  pills  daily,  each  con- 
taining  one  milligram,  which  wcve  gradually  increased  to  fifteeu.  lu  April, 
May,  and  Junc,  the  galvanic  stimulus  and  hydropathy  werc  associated  witli 
faradisation  ;  during  the  course  of  this  period,  especially,  the  most  remarkablc 
progress  was  made.  In  July  and  August,  at  JBagncres-de-LuQlion,  baths, 
douches,  and  energetic  massaye  (kneading). 


PERSONAL    OBSERVATIONS.  151 

persistedj  on  the  'zSth  and  39th,  without  being  so  strong  as  to  hinder 
M.  E —  from  remaining  up,  ont  of  bed_,  a  part  of  the  day. 

The  only  noticeable  peculiarity,  during  this  period  of  three  days, 
was  the  appearance  on  the  trunk  of  a  douhle  zona,  traces  of  which 
are  still  to  be  seen  (Nov.,  1873).  The  éruption  occupied  the 
whole  of  the  thorax,  from  top  to  bottom.  In  front,  we  saw;  1°, 
on  the  rîfjM,  a  first  group  of  vesicles  under  the  axilla  ;  a  second,  also 
hiteral,  in  the  vicinity  of  the  lower  margin  of  the  pectoralis  major  ; 
a  third,  médian,  situated  beneath  the  ensiform  process  ;  2°,  on 
the  left,  a  group  corres})onding  to  the  second  right  group,  and 
another  situated  to  the  left  of  the  médian  line,  at  an  equal  distance 
from  the  umbiHcus  and  the  lower  extremity  of  the  sternum. 
Behind,  there  existed  a  group  on  a  level  with  the  lower  angle  of 
the  scapula  ;  and  a  second,  more  latéral,  nearly  equi-distant  from  the 
latter,  and  the  crest  of  the  ilium.  It  appears  certain  that  this  zona 
was  not  accompanied  by  any  pains  localised  in  the  course  of  the 
nerves. 

Without  having  experienced  either  pains  or  formication,  on  the 
ist  October,  in  the  morning,  whilst  rising,  and  hardly  out  of  bed, 
Ch.  E, —  felt  his  legs  give  way  under  him,  and  he  fell  heavily  on 
the  ground.  Hence,  it  was  during  the  night-time,  between  Sept. 
3oth  and  Oct,  ist,  that  the  paraplegia  took  place.  The  patient  was 
replaced  in  bed.  It  seems  quite  established  that,  on  this  day,  he 
had  no  longer  any  fever.  The  paralysis  was  accompanied,  from  the 
outset,  with  marked  flaccidness  of  the  iuferior  extremities.  Their 
cutaneous  sensibility  was  never  modified  :  it  caunot  be  ascertained 
whether  they  were  cold  or  cyanosed.  Some  partial  movements  hâve 
always  persisted.  Thus  E —  has  always  been  able  to  extend  and  flex 
his  toes  ;  on  the  other  hand,  he  was  quite  incapable  of  raising  lus 
legs  completely  from  ofP  the  bed.  It  was  stated  that  the  emacia- 
tion  of  the  thighs  was  already  noticeable  a  few  days  after  the 
invasion. 

The  upper  extremities  hâve  not,  at  any  time,  been  seriously 
involved,  and  E —  has  always  been  able  to  use  his  hands,  either  for 
taking  food  or  to  hold  a  book.  No  disorder  has  ever  disturbed  the 
régulai  exercise  of  the  functions  of  bladder  and  rectum. 

The  period  of  rétrocession  began  to  set  in  a  very  short  time  after 
the  invasion.  Thus,  towards  the  1 5th  day,  E —  could  stand  erect 
by  resting  his  hands  on  adjacent  objects. 

The  actual  condition,  noted  the  lyth  Nov.,  1873,  gives  us  the 


152  PERSONAL    OBSERVATIONS. 

following  : — E —  can  stand  erect  and  even  take  some  steps  by 
leaning  with  both  bis  hands  on  tbe  shoulders  of  bis  servant.  Tbe 
inferior  extremities  are  emaciated  tlirougbout^  but  tbe  atropby  is 
particularly  évident  in  tbe  tbighs  whicb  are  flaccid,  and,  as  it  M'ere, 
flattened  from  before  backwards,  wbilst  tbe  calves  are  still  ratber  full 
and  fîrm.  Tbe  muscles  of  tbe  pelvis  seem  to  be  particularly  attacked. 
Tbus,  wben  E —  is  seated  be  cannot  flex  tbe  tbigbs  upon  tbe 
abdomen  ;  be  can  bardly  even  make  tbe  attempt.  Wben  lying  on 
bis  back,  it  is  quite  impossible  for  bim  to  raise  bis  trunk.  Wben 
tbe  patient,  sustained  in  tbe  uprigbt  position,  endeavours  witb  tbe 
belp  of  an  assistant  to  walk,  be  is  seen  at  every  step  to  roll  {Jianclier) 
excessively,  and  to  incline  tbe  trunk  greatly  first  to  one  side,  then 
to  tbe  otber  in  succession.  Tbe  gênerai  bealtb  remains  always 
good. 

We  saw  M.  E —  in  October,  1874;  tbe  motions  of  tbe  lower 
extremity  bave  regained  tbeir  normal  strengtb,  and  be  can  now  go 
tbrougb  ail  bodily  exercises  witbout  fatigue.  A  certain  degree  of 
comparative  emaciation  and  of  flaccidness  of  tbe  anterior  muscles  of 
tbe  left  thigb,  a  marked  tendency  of  tbe  trunk  to  incline  towards 
tbe  rigbt,  wbilst  standing  or  walking,  are  at  présent  tbe  sole 
vestiges  of  tbe  spinal  disease. 

Tbe  facts  wbicb  bave  just  been  detailed,  as  examples,  and  wbich 
migbt  easily  be  multiplied,  will  sufïice,  I  bope,  gentlemen,  to  render 
it  évident  tbat  certain  cases  of  acute  spinal  paralysis  observed  in  tbe 
adult,  are,  from  a  clinical  point  of  view,  quite  comparable  to  tbe 
spinal  paralysis  of  young  infants.  It  remains  to  be  determined  if, 
as  seems  probable,  tbe  spinal  lésion,  wbence  tbe  symptomatic  group 
lesults,  acknowledges  in  tbe  adult  tbe  strict  localisation  in  tbe 
anterior  cornua,  and  ail  tbe  otber  cbaracters  wbicb  distinguisli 
tbat  of  tbe  cbild.  Autopsy  bas  not  yet  given  its  final  décision. 
Tbere  is  a  void  bere  wbicb  cannot  fail  soon  to  be  fiUed.^, 

^  Clinically,  Ihe  case  published  by  my  bouse -surgeon,  M.  Gombault,  in  tbe 
*Arcbives  de  Pbyeiologie,'  Jan.,  1873,  p.  60,  unquestionably  approximates 
greatly  to  infantile  spinal  paralysis  ;  it  differs  from  it  in  some  respects,  bow- 
ever,  from  an  anatomo-patbological  point  of  view.  Tbe  motor-cells  were 
seriously  altered  in  tbe  régions  of  tbe  spinal  cord  corresponding  to  tbe  atropbied 
muscles;  but,  nowbere  in  tbe  anterior  eornua  were  limited  foci  observed^ 
causing  tbe  disappearance  of  entire  groups  of  nerve-cells,  and  producing 
fibroid  tbickening  of  tbe  interstitial  tissue,  wbicb  appears  to  be  a  constant 
eharacter  of  tbe  spinal  lésion  proper  to  tbe  atrophie  paralysis  of  young  infants. 


PATHOLOGICAL   ANATOMY   AND    PHTSIOLOGY.  153 

II. 

I  tliink  it  useful  to  recur,  to-day,  to  several  points  relative  to  tlie 
^atJiolog'ical  anatomy  and  ]i]i2jsiology  of  infantile  spinal  parali/sis. 
I  shall  tlius  hâve  an  opportunity  of  noticing  and  making  use  of 
several  works  which  hâve  appeared  upon  this  subject  since  the 
publication  of  the  first  investigations  undertaken  at  La  Salpêtrière. 
Thèse  first  researches  concerning  the  inmates  of  the  asylum — 
persons,  to  wit,  who  had  most  frequently  succumbed  a  very  long 
time  after  the  period  of  childhood — were  unquestionably,  on  that 
account,  made  in  comparatively  unfavorable  circumstances.  They 
hâve,  however,  enabled  us  already  to  establish  some  fundamental 
data  which  later  investigations,  carried  on  under  happier  condi- 
tions, namely,  in  young  subjects  who  had  died  at  a  period  con- 
tiguous  to  the  invasion  of  the  disease,  hâve  completed  in  some 
respects  but  hâve  not  essentially  altered.  This  will  be  évident,  I 
think,  from  the  short  account  that  follows. 

1°.  What  especially  characterises,  anatomically,  the  spinal  lésion 
of  infantile  paralysis  is  the  strict  systematic  locaHsation  of  the 
altérations  in  the  anterior  cornua  of  the  grey  substance,  and,  more 
particularly,  in  the  région  of  the  cornua  occupied  by  the  great  gan- 
glionic  cells,  which  are  called  motor.  Nothing,  up  to  the  présent, 
has  been  found  to  contradict  this  proposition,  estabHshed  from  the 
beginning  of  our  researches.^ 

The  altération  in  question,  and  on  this  point  we  hâve  not  failed 
to  lay  stress,  shows  itself  occasionally  exactly  confined  to  only  one 
or  two  of  the  distinctly  circumscribed  oval  groups  which,  as  for 
instance  in  the  lumbar  enlargement,  are  constituted  by  thèse  ceUs 
associating  together  (fig.  lo).  Those  are,  so  to  say,  the  primary 
foei  of  the  affection,  for,  if  the  lésion  extend  beyond,  it  appears  to 
radiate  around  the  cell-groups  as  around  a  centre.  Only  when  the 
altération  is  most  intense,  and  then  merely  hère  and  there  in  a  few 
places,  the  grey  cornu  is  invaded  throughout  its  whole  extent 
■transversely  (fig.  9).  It  is  the  rule,  in  such  cases,  that  the  white 
columns,  especially  the  anterior  and  latéral  columns,  shall  présent 
in  the  région  where  the  grey  cornu  is  so  gravely  attacked,  a  kind  of 

^  Thèse  views  concerning  the  part  played  by  the  altération  of  nerve-cells  in 
the  anterior  cornua  in  the  pathogeny  of  infantile  paralysis  and  progressive 
spinal  amyotrophics  were  detailed  in  a  lecture  delivered  by  me  at  the  Salpê- 
trière in  June,  1868. 


154  PATHOLOGIOAL    ANATOMY   AND    PHYSIOLOGY. 

emaciotion,  of  atrophy,  witli  more  or  less  marked  decrease  iu  every 
diameter;  but  this  evidently  secondary  lésion  of  tlie  white  columns 
is  iiot  iiecessary.  It  may  be  déficient  (fig.  9),  and  cannot,  tlierefore, 
take  its  place  in  the  front  rank  among  the  cbaracteristics  of  the 
spinal  lésion  peculiar  to  infantile  paralysis. 

The  very  remarkable  localisation  of  the  lésions  in  the  area  of  the 
cell-group  has  long  led  me  to  adinit^  as  a  probable  hypothesis,  that 
the  morbid  process  first  occupies  the  nerve-cell,  extcnding  afterwards 
to  the  neuroglia.  How,  in  fact,  is  it  possible  othervvise  to  explain 
the  very  striking  circumscription  of  the  altération  to  the  immédiate 
vicinity  of  the  ganglionic  éléments  ?  I  am  aware  of  no  serions 
argument  which  has,  till  now,  been  advanced  against  this 
hypothesis. 

1°.  The  lésion  of  the  anterior  horns,  in  cases  of  old  standing, 
such  as  those  which  served  for  our  researches,  generally  consists,  so 
far  as  the  nerve-cells  are  concerned,  of  a  more  or  less  advanced 
scierons  airophij.  The  ganglionic  éléments  of  a  whole  group,  or  of 
a  whole  région,  when  the  altération  is  carried  to  its  utmost,  may 
even  hâve  disappeared  altogether,  leaving  no  trace.  As  to  the 
neuroglia,  it  exhibits  signs  of  connective  hyperplasia  with  multi- 
plication of  nuclcar  éléments  and  formation  of  fibroid  tissue,  often 
very  dense — manifest  évidences  of  the  previous  existence  of  irritative 
action. 

Sfeill,  as  we  hâve  mentioned  elsewhere,  the  observations  noted  at 
the  Salpôtrière  had  only  allowed  us  to  examine  the  rewinant«  of  a 
long  extinct  morbid  process.  In  présence  of  the  évidence  which 
tliey  furnished,  we  could  only  seek  to  construct,  by  a  kind  of 
exegesis,  the  lirst  phases  of  the  process.  Undoubtedly,  the  lésions 
of  the  neuroglia  presented  us  with  incontestable  traces  of  their 
inflammatory  origin.  But  had  there  been  hère  at  work,  formerly, 
a  hyperplasUc  myelitis  loithout  dissociation  of  tissue  ;  or,  on  the  con- 
trary,  a  destructive  myelitis,  toitli  softenivg  ?  The  problem  was  one 
almost  impossible  of  solution. 

Hère  chiefly  lies  the  interest  of  the  important  observations  of 
MM.  Damaschino  and  Roger.^  Thèse  authors  hâve  had  occasion 
to  make  a  post-mortem  examination  in  the  case  of  two  children  who 
succumbed,  one  two  monthB,  and  the  other  six  months,  aft^ 
the  outbreak  of  the  disease,  and  they  hâve  remarked  in  both  caises 
that,  in  the  most  seriously  alterred  parts  of  the  cord,  the  lésions 

'  Loc.  cit. 


PATHOLOGICAL    ANATOMY    AND    PHYSIOLOGY.  155 

(localisée!  according  to  rule  in  oue  of  the  grey  antcrior  cornua)  con- 
sisted  of  recl  inflammatonj  ramolUssement  with  vascular  iujection, 
production  of  granular  bodies,  &c.  Above  and  below  thèse  points 
the  altération  could  still  be  followed  for  a  certain  distance  in  the 
grey  substance,  but  gradually  lessening  it  shortly  was  only  repre- 
sented  by  the  multiplication  of  nuclear  éléments,  and  by  a  vascular 
injection  which  was  especially  évident  in  the  immédiate  vicinity  of 
the  groups  of  nerve-cells. 

Thèse  observations  establish,  as  may  be  seen,  that  red  ramollis- 
sement must  be  enumerated  among  the  spinal  lésions  of  infantile 
paralysis.  But  nothing  has  hitherto  demonstrated  that  this  is 
an  obligatory  accompaniment.  It  is  even  highly  probable  that, 
just  as  sometimes  happens  in  common  central  acute  myelitis,  the 
altérations  of  the  spinal  cord  in  infantile  paralysis  may  reach  their 
greatest  intensity,  and  even  détermine  the  gravest  trophic  muscular 
lésions  at  the  periphery,  without  the  occurrence  of  dissociation 
of  connective  and  nerve  éléments,  otherwise  called  ramollissement  J 

Another  interesting  fact,  rendered  évident  by  the  observations  of 
MM.  Roger  and  Damaschino,  is  this,  namely,  that  the  altération  of 
tlie  nerve-cells  is  marked  by  atrophy,  with  excessive  pigmentation 
of  thèse  éléments.  The  scierons  lésion,  mentioned  in  our  own 
observations,  would  therefore  be  a  subséquent  phenomenon.^ 

'  Cliarcot,  '  Archives  de  Physiologie,'  1872  ;  Janvier-rcvrier  ;  ïïayem,  idem, 
1874,  p.  603. 

-  This  is  Ihe  place  to  locall  the  principal  modes  of  altération  to  which  the 
nerve-cells  of  the  grey  aiiterior  coiniia  of  the  spinal  cord  are  susceptible. 

1°.  I  would,  first,  mention  the  often  enormous  tuméfaction  which  thèse  cells 
oceasionaUy  undergo,  aud  which  I  believe  I  was  the  first  to  recoguise,  so  far, 
at  least  as  the  card  is  couoerned  (Soc.  de  Biologie,  1872).  Tlie  body,  volumin- 
0U8  and  as  if  swollen,  is  at  once  turbid  aud  opalescent.  The  prolongations  are 
thicker  than  in  the  natural  state,  and  seemingly  writhen.  I  hâve  comparcd 
tliis  altération  of  the  ncrvc-cells  of  the  spinal  cord  to  the  hypcrtrophy  which, 
under  the  influence  of  certain  irritative  processes,  is  presented  by  the  axis 
cylinder,  either  in  the  oerebro-spinal  centre  or  in  the  peripheral  nerves  (Fig. 
10,  b). 

2°.  Somc  authors  hâve  dcscribed,  in  the  nerve-cells  of  ihe  encephalou,  a 
multiplication  of  nuclci  (JoUy)  which  they  regard  as  the  sign  of  au  irritative 
process.  M.  Leyden  states  that  ho  remarked  the  same  thing  with  respect  to 
the  ganglionic  cells  of  the  cord.  But,  it  is  important  to  observe  that,  in 
certain  régions  of  the  enccphalon,  and  in  the  great  sympathetic  system,  the 
présence  of  two  nuclei  in  a  nerve-cell  is  a  fact,  one  which  is  uudoubtedly  rare,  but 
which  yct  Ls  sceu  under  normal  conditions,  irrespective  of  any  trace  of  irrita- 
tive action  ;  no  prolifération  of  the  ueivc-cell,  for  instance,  is  observed  corre- 


156 


PATHOLOGICAL   ANATOMY    AND   PHYSIOLOGY. 


3°  The  spinal  lésion,  wliose  principal  features  hâve  just  been 
sketched,  is  constant  in  infantile  paralysis  ;  that  is  a  fundamental 
fact  which  I  hâve  already  intimated  in  my  lectures  of  1868,  and 

sponding  to  the  prolifération  of  tlie  cellular  éléments  of  the  connective  tissus. 
In  short,  the  différent  altérations  whicli  the  cells  undergo  owiug  to  inflam- 
mation, besides  the  tuméfaction  mentioned  above,  are  ail,  anatomically  speaking, 
atrophie  or  degenerative  (lig.  11,  c,  d,  e). 


FiG.  II. — Nerve-cells  of  the  anterior 
cornua  of  the  spinal  cord. 

A.  Normal  state. 

B.  Hypertrophie  state. 

c.  Pigmentary  altération. 

D.  Last  stage  of  pigmentary  change. 

E.  Cell  stricken  with  scierons  atrophy. 
p.  Vacuolar  altération. 


E 


3°.  I  will  mention,  in  passing,  the  altération,  termed  vacuolar,  of  the  nerve- 
cells  of  the  anterior  cornua.  I  hâve  oft  times  met  with  it  in  cases  where 
the  neuroglia  presented  unequivocal  signs  of  inflammation  in  the  neighbour- 
hood.  I  hâve  not  yet  been  able  to  conviuce  myself  that  this  altération  is  not 
an  artilîcial  product  (Fig.  11,  p). 

4°.  I  will  dwell,  at  more  length,  on  the  altération  of  the  spinal  nerve-cells, 
known  as  pigmentary.  It  is  a  normal  fact,  so  to  speak,  that  ia  old  âge  thèse 
cells  shall  be  ûUed  and  distended  by  an  often  enormous  quantity  of  pigment. 
Is  this  a  fact  of  no  conséquence  whatever  as  regards  functional  exercise  ? 
Should  we  not  rather  attribute,  pai'tially  at  least,  to  this  senile  modification  of 
the  cell  the  motor  enfeeblement  and  the  altérations  of  the  muscles  of  the  limbs 
which  show  themselves,  as  it  were  necessarily,  at  a  certain  period  of  life  ? 

However  this  be,  the  accumulation  of  pigment  in  a  spinal  nerve-cell  is 
not  sufficient  in  itself,  whatever  its  intensity,  to  characterise  a  profouud  lésion 
of  the  organism.  But  there  is  superadded  in  pathological  cases  proper,  a  genuine 
atrophy,  whose  phases  hâve  been  well  described  by  L.  Clarke.  In  the  first 
degree  of  this  altération,  the  cell  diminishes  in  volume,  and  the  transparent 
part  of  the  body  becoraes  more  and  more  reduced  ;  in  the  second,  the  pro- 
longations atrophy  in  their  turn,  whilst  the  body  assumes  a  globular  form  ; 
soou  after  the  prolongations  are  only  represented  by  short  slender  filaments. 
Finally,  they  disappear,  in  the  last  stage.     Concomitantly,  the  nucleus  of  the 


PATHOLOGICAL   ANATOMT    AND    PHYSIOLOGY.  157 

which  ail  the  observations  since  published  in  France  or  abroad,  and 
they  are  numerous  to-day_,  go  to  confirm.  Amongst  thèse  corrobo  ■ 
rating  observations,  to  mention  only  the  most  récent,  I  would  cite 
those  which  hâve  been  published  in  Germany  by  HH.  Reckling- 
hausen,  Rosenthal  (of  Vienna),  and  Eoth  (of  Bâle).! 

I  strove,  in  addition,  at  that  period,  to  establish  that  the  lésion 
in  question  ought  to  be  considered  as  an  initial,  primavy  lésion, 
dominating,  as  it  were,  the  whole  morbid  drama.  It  could  not,  in 
fact,  be  admitted  that  it  was  a  conséquence  of  the  functional  inertia 
of  the  members  stricken  with  paralysis  ;  for  it  has  nothing  in 
common  with  the  peculiar  altération  of  the  spinal  cord,  which 
supervenes  after  amputations  of  old  standing,  and  which  had  already 
been  very  thoroughly  studied  by  MM.  Clarke,  Vulpian,  and  Dick- 
inson.2  Again,  the  hypothesis  which  would  place  the  starting 
point  of  the  phenomena  at  the  periphery,  either  in  the  muscles  or 
in  the  nerves,  would  be  very  complicated,  embarrassed,  and  unsup- 
ported  by  analogy  ;  whilst  the  opposite  hypothesis,  on  the  contrary, 
besides  the  support  it  dérives  from  the  history  of  common  central 
acute  myelitis  also  counts  the  results  of  expérimentation  upon  ani- 
mais on  its  side.  Por,  by  this  method,  M.  Prévost  has  shovvn  that 
a  lésion  aiîecting  the  central  parts  of  the  cord  détermines  muscular 
lésions  very  similar  to  those  which  are  observed  in  the  paralysis  of 
young  children.'^ 

The  opinion  which  I  endeavoured  formerly  to  advance  has  not, 

cell  becomes  atrophied.  This  'pigme7itary  atrophy,  which  conduces  to  the  entire 
destruction  of  the  cell,  is  seen  in  connection  with  irritative  processes,  primarily 
developed  in  the  adjacent  neuroglia  ;  or  else,  it  exists  in  au  isolated  fashiou, 
independent  of  any  lésion  of  the  neuroglia,  as,  for  instance,  in  certain  forms  of 
progressive  muscular  atrophy  or  of  bulbar  paralysis  (fig.  ii,  c,  d). 

5°.  Finally,  a  last  form  of  altération  of  the  motor  nerve-cell  is  that  some- 
times  desiguated  by  the  name  of  sclerosis  or  scierons  atrophy.  The  cell  is 
diminished  in  volume,  sometimes  to  a  considérable  extent.  It  is  shrunkeu,  as 
it  were,  and  more  or  less  rounded  ;  or,  on  the  contrary,  lengthened  out.  The 
prolongations  are  short,  dried  up,  or  absent.  The  cell-body  is  opaque,  brilliant  ; 
the  nucleuB  is  small,  irregular,  and  shrunken.  I  am  not  aware  if  this  altération 
is  always  preceded  by  the  lésions  of  pigmentary  atrophy,  or  if  it  may  be  primary. 
It  is  frequently  met  with  in  cases  of  spinal  amyotrophy  connected  with  a  well- 
marked  irritative  process  (fig.  ii,  e)  ('Cours  de  la  Faculté,'  1874). 

'  Roth,  "Anatom.  Befund  bei  spinaler  Kindérlàhmung,"  in  Virchow's 
'  Arehiv,'  1873,  t.  Iviii,  p.  273. 

-  Vulpian,  'Archives  de  Physiologie,'   1S68,  p.  443;  idem,  1869,  p.  675. 

^  Prévost,  'Société  de  Biologie,'  séance  du  14,  avril,  1872. 


158  PATHOLOGICAL    PHYSIOLOGY. 

fls  you  perceive,  met  witli  any  serious  objection;  it  appears,  in 
fact,  to  be  l'atlier  generally  clisseminated  at  présent.  Coiisequently, 
I  believc  that,  in  accordance  witli  tliis,  I  can  keep  to  the  tlieory 
wliicli  T  at  tlie  time  proposed  in  référence  to  the  concaténation  of 
])benoinena.  The  nerve-cells  are,  ex  hypotheù,  the  priinary  seat  and 
starting  point  of  the  irritative  process,  and  there  we  fînd  produced 
an  acnte  anterior  j^arenchymatous  icphro-myelïtls}  The  process 
rapidly  sprcads,  bit  by  bit^  to  the  adjacent  connective  tissue, 
without,  howcver,  exceeding  the  hmits  of  the  area  of  the  anterior 
cornua.  Whilst^  under  the.  influence  of  this  morbid  incitation,  the  cell 
passes  tlirougli  the  différent  phases  of  atrophy  capable  of  issuing  in 
complète  destruction,  the  neuroglia  itself  reacts  after  its  manner, 
and  becoraes  inflamed.  The  phlegmasic  action  may  hère,  in  the 
most  altered  points,  procecd  even  to  the  formation  of  a  focus  of  red 
softening. 

However  this  may  be,  to  thèse  abruptly  developed  altérations  ail 
the  phenomena  of  the  disease  are  attached  ;  namely,  first,  the  initial 
fébrile  action,  tlien  the  whole  séries  of  symptoms  which  soon  succeed. 
Motor  paralysis,  marked  by  suppression  of  muscular  tone  and  of 
the  other  modes  of  reflex  activity,  may  be  considered,  according  to 
the  physiological  view,  at  présent  prévalent  as  a  very  simple  con- 
séquence of  the  disorganisation  from  which  the  apparatus  of  motor 
nerve-cells  is  suffering.  Erom  this  same  lésion  of  the  ganglionic 
éléments  are  also  certainly  derived  the  rapid  atrophy  of  the  paralysed 
muscles,  and  the  modifications  of  electric  contractility  which  form 
its  prélude  ;  but  the  pathogenetic  modus  which  should  be  hère 
arraigned  is  not  yet  well  known.  It  is  readily  admitted  that  the 
centrifugal  nerves,  which  hâve  their  origin  in  the  affected  parts  of 
the  spinal  cord,  conduct  themselves  like  the  peripheral  end  of  a 
divided  nerve.  They  would  undergo  the  several  phases  of  destruc- 
tive altération  (which  MM.  Neumann,  Eanvier,^  Vulpian,  and 
Eichhorst^  hâve,  in  latter  days,  studied  with  such  great  care  in  con- 
nection with  expérimental  nerve  lésions),  and  the  observed  loss  of 

'  H.  Kussmaul  bas  proposed  tlie  term  poUo-nii/elitis  anterior  acutissima  to 
designate  the  spinal  lésion  of  infantile  paralysis  (loc.  cit.,  No.  i,  p.  3).  I 
believe  Lephro-myelitis  is  préférable,  and,  in  référence  to  this  subject,  I  can 
invoke  the  potent  authority  of  M.  Littrc  (rk^pa,  cinis.  Plut. — Ts^pàioç, 
■einereiis,  Jîlian). 

"  Ranvier,  'Comptes-rendus  de  l'Académie  des  Sciences/  1872,  1873. 

2  Eiclihorst,  '  Virchow's  Archiv,' 59  Bd.,1874. 


ACUTE    AÎIYOTROPHIES    FROM    DIFFUSE    SPINAL    LESIONS.     159 

faradaic  coiitractility,  as  well  as  the  atrophy  of  the  muscular  buudles, 
would  supervene  as  a  conséquence.  It  must  not  be  forgotten  that 
this  is  as  yet  ouly  an  hypothesis,  tbough  it  is,  indeed^  a  bighly 
plausible  one  ;  and  it  is  important  to  remark,  in  particular,  tbat  the 
State  of  tbe  peripheral  nerves,  in  the  first  weeks  which  follow 
the  invasion  of  infantile  paralysis,  bas  not  yet  been  observed  de 
visu. 

As  to  the  constantly  noted  absence  of  lastiug  disturbance  of  the 
cutaneous  sensibility,  of  paralysis  of  the  rectum  or  bladder,  of 
cutaneous  or  viscéral  trophic  disorders,  this  holds,  as  you  know,  an 
eminent  position  amongst  the  characteristics  of  infantile  paralysis 
and  it  contributes  largely  to  distinctly  separate  this  affection  from 
the  différent  forms  of  diffuse  acute  myclitis.  It  is  physiologically 
correlated  to  the  strict  localisation  of  the  spinal  lésion  within  the 
area  of  the  anterior  cornua  of  grey  matter.  Hère,  by  the  opération 
of  the  disease,  in  the  central  parts  of  the  spinal  cord,  a  délicate  and 
always  successful  experiment  is  made,  which  demonstrates  that  the 
anterior  grey  cornua  are  not  necessary  to  the  transmission  of  sen- 
sory  impressions,  and  that  they  bave  no  direct  influence  over  the 
motions  of  the  bladder  and  rectum,  nor  upon  the  nutrition  of  the 
skin  or  of  the  genito-urinary  organs. 

m. 

If  the  views  which  bave  just  been  detailed  are  sound,  it  should 
follow  that  every  acute  inflammatory  lésion  of  the  spinal  cord, 
whatever  may  be  its  origin,  would  necessarily  produce,  like  infantile 
paralysis,  motor  paralysis  with  rapid  atrophy  of  the  paralysed 
muscles,  provided  that  the  express  condition,  so  often  prominently 
put  forward  already,  be  hère  présent,  namely,  the  acide  atrophie 
lésion  of  the  motor  nerve-cells.  On  the  other  hand,  the  phenomena 
to  which  I  bave  just  called  attention,  and  which  are  regularly 
déficient  in  the  symptomatology  of  infantile  paralysis  because  of 
this  very  systematic  circumscription  of  the  altération  to  the  area  of 
the  anterior  cornua, — thèse  phenomena,  I  say,  ought,  on  the  con- 
trary,  to  be  met  with  in  différent  degrees  in  ail  the  other  acute  forms 
of  the  spinal  affection,  because  they  ail  ackuowledge  as  their  sub- 
stratum  lésions  which  are  more  or  less  diffused. 

So,  in  reality,  things  do  happen,  as  we  fiud  testified,  for  example, 
by  the  history  of  one  of  the  commonest  as  well  as  one  of  the 


GENEEALTSED  ACUTE    CENTEAL  MYELITIS. 

gravest  of  spinal  diseases  of  tlie  adult.  I  refer  to  generalised  acute 
central  myelitïs?-  The  lésion  hère  is  generally  apparent  to  the 
naked  eye  as  red  ramolHssement.  But  matters  do  not  always  pro- 
ceed  so  far,  and  l)oth  the  connective  and  nerve-elements  may  show 
themselves  greatly  altered,  without  having  undergone  dissociation.^ 
However  this  be,  it  occupies  the  central  régions  of  the  spinal  cord, 
especially  the  grey  substance,  and  tends  to  invade  a  great  portion 
of  the  height  of  the  nerve-column  ;  so  that,  for  instance,  the  dorsal 
and  lumbar  région  will  be  simultaneously  attacked  throughout  their 
whole  length.  In  the  grey  substance,  it  involves  the  anterior  grey 
cornua,  and  consequently  the  motor  cells,  but  it  does  not  confine 
itself  to  this,  for  it  attacks  also  the  posterior  grey  cornua  and  the 
commissures.  Finally,  it  likewise  always  spreads,  hère  and  there, 
irregularly  over  the  différent  white  columns. 

The  invasion  takes  place  often  abruptly  and  it  may  be  mavked, 
as  in  infantile  paralysis,  by  a  fébrile  state  of  more  or  less  intensity. 
If  the  two  affections  be  compared,  with  regard  to  local  symptoms, 
it  will  be  remarked  that  several  are  common  to  both.  Others 
belong  only  to  diffuse  acute  myelitis.  The  common  symptoms  are  : 
motor  paralysis  with  complète  flaccidness  ;  precocious  diminution  of 
faradaic  contractility, — observed  in  several  cases  of  myelitis  from  the 
first  week  f  and,  lastJy,  rapidly  developed  atrophy  of  the  muscles. 
The  theory  would  indicate  that  thèse  dépend  on  the  altération  of  the 
anterior  grey  cornua.  On  the  other  hand,  new  superadded  sym- 
ptoms only  belonging  to  diffuse  myelitis  reveal  the  participation  of 
other  lésions  of  the  cord.  Thèse  are  :  more  or  less  marked  altéra- 
tions of  sensibility,  and  particularly  a  more  or  less  intense  cutaneous 
anassthesia  of  the  paralysed  merabers  ;  paralysis  of  the  bladder  and 
rectum  ;  émission  of  alkaline,  purulent  urine  ;  finally,  the  formation 
of  bed-sores  not  only  in  the  sacral  région,  but  also  over  ail  points 
of  the  paralysed  member^subjected  to  pressure  of  some  duration. 

Thèse  bed-sores  which,^like  the  other  phenomena  already  men- 
tioned,  are  absolutely  déficient  in  infantile  paralysis,  appear  as  a 

1  The  vast  altérations  whicli  may  affect  the  muscles  of  the  paralysed  mem- 
bers  in  acute  central  myelitis  hâve  beeu  already  pointed  out  by  Rokitansky 
('Lehrb.  des  Path.  Anat.,'  le  Bd.,îi855,  p.  329;  2e  Ed.,  1856,  p.  228). 

"  See  my  observations  on  the  histology  of  acute  myelitis  ('Archives  de 
Physiologie/  1872,  Janv.,  Fev.)  and  those  of  M.  Hayem  on  the  same  subject, 
(idem,  1874,  p.  603). 

•^  Observations  of  H.  Mannkopf,  '  Amtlieh.  Bericlit  ûber  die  Versammlung 
Deutsch  Naturforscher  und  Aerzte  zu  Hannover,'  p.  251,  1S66. 


H^MATOMYELIA.      TEAUMATIC  LESIONS.  161 

common  fact  in  acute  généralisée!  myelitis.  It  is  known  that  they 
are  produced,  with  singular  rapidity,  four^  six,  or  ten  days  after  the 
invasion  of  the  first  symptoms,  and  that  they  contribute  powerfully 
to  détermine  the  fatal  issue. 

Hœmatomyelïa  or,  in  other  words,  intra-spinal  hœniorrhage,  is 
open  to  similar  comment.  In  a  number  of  cases,  in  fact,  its  sym- 
ptomatic  history  fuses,  in  ail  points,  as  it  were,  with  that  of  acute 
generalised  myelitis.  Thus,  to  mention  merely  the  faradaic  con- 
tractility,  this  has  been  seen  to  disappear  on  the  i4th,^  and  on  the 
çth  day  32  and,  again,  large  bedsores  often  rapidly  show  themselves 
on  the  nates.  Intra-spinal  hsemorrhages,  as  M.  Hayem''  and  I, 
myself,^  bave  striven  to  establish  would,  in  fact,  appear  to  be  only 
a  kind  of  ejnphenomenon  of  acute  central  myelitis.  It  seems 
certain  that,  almost  always,  the  effusion  of  blood  takes  place  there 
in  the  midst  of  parts  previously  modified  in  structure  owing  to  the 
présence  of  inflammation. 

Traumatic  lésions  of  the  spinal  cord,  whether  they  resuit  from 
fracture  of  the  vertébral  column  or  from  a  wound  deaJt  with  a 
sharp  instrument,  may  also  détermine  the  occurrence  of  acute  amyo- 
trophy  with  ail  its  accompaniments,  in  a  word,  the  formation  of 
bedsores  of  rapid  development.  The  spinal  changes,  in  cases  of 
traumatic  injuries  are,  at  least  at  first,  those  of  acute  transverse 
myelitis  :  that  is,  we  shall  hâve  inflammatory  lésions  which,  involv- 
ing  both  the  grey  axis  and  the  white  columns,  only  occupy  a  small 
extent  of  the  cord,  in.  height.  But  they  often  extend  very  rapidly 
beneath  the  point  primarily  affected,  down  to  the  extremity  of  the 
lumbar  enlargement,  for  instance,  if  the  lésion  hâve  affected  a  point 
in  the  dorsal  région.  The  extension  in  question  takes  place  in  the 
"white  columns,  according  to  a  well  known  law,  along  the  latéral 
fascicles  ;  whilst  in  the  grey  substance,  the  parts  affected  are  the 
columns  formed  by  the  anterior  coruua.  This  descending  exten- 
sion of  transverse  spinal  lésions  is  not  a  mère  supposition  or  view  ; 
I  hâve  quite  recently  observed  it  distinctly  on  examining  sections 
taken  from  a  case  of  acute  transverse  myelitis,  to  which  I  shall 
again  refer.     This  alone  enables  us  to  understand,  as  I  hâve  else- 

^  Observatioû  of  Levier,  '  Beitrâge  zur  Patliologie  der  Ruckenmarks  Apo- 
plexie,' "  Inaug.  Dis.,"  Bern,  1864. 

"  Observation  of  Duriau,  '  Union  Médicale,'  1859,  ^-  i'  P-  3°^- 
^  Hayem,  'Des  Hemorrhagies  intra-rachidieuues,'   1S72,  p.  138. 
■*  Charcot,  '  Leçons  de  la  Salpêtrière,'  1870. 
VOL.   II.  11 


162  TRAUMATIO  SPINAL  LESIONS. 

where  pointed  out,^  liow  a  spinal  lésion  appareiitly  limited  to  a  cir- 
cumscribed  part  of  tlie  dorsal  région  may  détermine,  in  tlie  motor- 
paralysed  lower  extremities,  acutc  atrophy  of  the  muscles  and,  in  a 
Word,  ail  the  phenomena  wliicli  dérive,  as  the  physiological  analysis 
of  infantile  paralysis  proves,  from  acute  atrophy  of  the  motor  nerve- 
cells. 

^  Charcot,  'Lectures  on  Diseases  of  the  Neivous  System/  vol.  i,  p.  51, 
note  ^ 


■>. 


LECTURE  XI. 

CHRONIC    SPINAL    AMYOTROPHIES.     PROTOPATHIC    SPINAL 
PROGRESSIVE    MUSCULAR    ATROPHY    (DUCHENNE-ARAN 

TYPE). 

SuMMARY. — Cliuical  varieties  of  cases  designated  under  the  name  of 
progressive  muscular  atrophy  [spinal  progressive  muscular 
atrophies).  UniformMi/,  in  thèse  cases,  of  the  spinal  lésion 
which  afjects  the  anterïor  cormia  ofthe  grejj  suhstance. 

Studij  of  protopathic  spinal  progressive  vmscular  atrophy 
as  typical  of  the  group  :  simple  spinal  lésion.  Chronic  deutero- 
pathic  spinal  amyotrophies.  The  lésion  of  motor  nerve-cells  is 
hère  consécutive  ;  it  is  superadded  to  a  %pinal  lésion  of  variable 
position.  Account  of  the  principal  spinal  affections  îvhich  may 
produce  deuteropathic  progressive  amyotrophy  :  hypertrophie 
spinal  pachymeningitis  ;  sclerosis  of  posterior  cohimns  ;  chronic 
central  myelitis  ;  hydrornyelia  ;  intra-spinal  tumoitrs s  dis- 
seminated  sclerosis  ;  symmetrical  latéral  sclerosis. 

On  protopathic  spinal  progressive  muscular  atrophy  in 
particîUar  {I)uchenne-Aran  type).  Symptoms  :  individual 
atrophy  of  the  muscles,  functional  disorders  ;  prolonged 
persistence  of  faradaic  contractility  ;  fihrillary  tremors  ; 
paralytic  déformations,  or  déviations,  "griffes."  Modes  of 
invasion.  Eliology  :  hereditary  transmission,  cold,  traumatic 
injuries. 

Pathological  anatomy.  Lésions  of  the  cord  ;  altération 
limited  to  the  anterior  cornua  of  the  grey  suhstance  {^nerve-cells, 
neuroglia).  Lésions  of  the  nerve-roots  and  of  the  peripheral 
nerves.     Muscular  lésions  and  their  nature. 

I. 

Gentlemen, — I  propose,  in  the  lectures  wliich  are  to  follow,  to 
dévote  some  discussion  to  the  history  of  chronic  spinal  amyotrophîes. 


K 


164  PEOTOPATHIO  SPINAL  AMTOTROPHY. 

The  affections  comprised  under  this  désignation  are^  even  yet,  often 
confounded,  in  practice,  under  the  common  name  of  inogressive 
muscular  atrophy.  Pathological  anatoiny,  liowever,  lias  long  since 
establislied  that  we  bave  hère  no  homogeneous  group. 

In  reality,  the  spinal  lésions  whicli  are  to  be  met  with  in  cases 
wbicli,  in  practice^  receive  this  name  of  progressive  muscular 
atrophy  are  very  various.  Tliey  possess,  however^  in  common,  a 
peculiar  feature,  wliich  constitutes,  as  it  were,  tlie  fundamental 
anatomical  character  of  the  group  ;  tliis  is,  the  lésion  ot  the  anterior 
cornua  of  the  grey  substance,  and,  to  be  more  explicit,  the  atrophie 
altération  of  the  motor  cells  of  the  région.  We  find  hère,  in  some 
degree,  the  reproduction  of  what  we  saw  in  connection  with  acute 
spinal  amyotrophies.  The  spinal  lésion,  however,  in  the  cases  which 
are  about  to  engage  our  attention  is  no  longer  evolved  after  the  acute 
mode,  but,  on  the  contrary,  proceeds  in  a  chronic  subacute  manner, 
and  to  this  circumstance  are  due  the  considérable  différences  which, 
in  spite  of  more  than  one  analogy,  are  observable  in  the  succes- 
sion of  symptoms. 

A.  You  doubtless  remember,  gentlemen,  that,  in  studying  acute 
spinal  amyotrophies,  we  took  as  our  objective  point  a  regular  type, 
uamely,  infantile  parali/sis,  where  the  spinal  lésions  are  systemati- 
cally  limited  to  the  anterior  cornua  of  the  grey  substance.  A  type 
of  the  same  kind  shall  serve  as  our  guide  in  the  history  of  chronic 
spinal  amyotrophies.  In  reality,  a  lésion  exactly  limited  to  the 
anterior  régions  of  the  grey  matter,  and  leaving  perfectly  intact  ail 
the  other,  white  and  grey,  substance  of  the  spinal  cord,  constitutes 
the  anatomical  substratum  in  a  certain  form  of  progressive  muscular 
atrophy  which  nearly  corresponds  clinically  to  the  common  type,  as 
described  by  Cruveilhier,  Duchenne  (de  Boulogne),  Aran,  and 
which,  with  your  permission,  we  shall  qualify  as  protopathie 
spinal. 

The  constitution  of  this  protopathic  form  of  spinal  muscular 
atrophy,  which  in  some  sort,  I  repeat,  reproduces  infantile  paralysis 
in  a  chronic  guise,  is  comparatively  very  simple.  Thus,  the 
anatomo-pathological  élément  is  represented  : 

1°.  In  the  cord,  by  a  lésion  systematically  limited  to  the  grey 
anterior  cornua;  the  altération  of  the  great  nerve  cells  being  like- 
wise  a  necessary  condition,  sine  qud  non,  and  sometirnos  the  only 
observable  lésion  ;  2°,  in  the  motor  roots  and  peripheral  motor 
nerves,  by  a  more  or  less  marked  atrophy,  as  a  conséquence  of  the 


DEUTEROPATHIC  SPINAL  AMYOTEOPHIES.       165 

spinal  lésion  ;  3°,  lastly,  in  the  corresponding  muscles^  by  trophic 
lésions  which  we  shall  hâve  to  pass  in  review,  and  whence  pro- 
ceeds,  properly  speaking,  tlie  entire  symptomatology  of  the 
affection. 

B.  Matters  are  more  complicated  in  a  second  group  of  chronic 
spinal  amyotrophies,  which,  by  way  of  distinction,  I  shall  designate 
by  the  name  of  deuterojmthlc.  Hère,  indeed,  the  lésion  of  the  an- 
terior  cornua  and  of  the  nerve-cells  is  necessarily  présent  also  ;  but 
it  is  only  second  in  date^  and  consécutive  in  ail  cases.  The  original 
lésion  still  occupies  the  spinal  cord,  but  it  is  developed  exteriorly  to 
the  grey  substance,  and  it  is  only  secondarily  and  by  extension  that 
the  latter  has  been  invaded  in  its  turn.  When  this  invasion  has 
taken  place,  then,  indeed,  the  same  séries  of  consécutive  phenomena 
follows  from  it,  and  progressive  atrophy  of  the  muscles  in  particular  ; 
however,  the  amyotrophic  symptoms  are  now  found  to  be  inter- 
mingled  with,  or  rather  superadded  to,  those  of  the  primary  spinal 
disease.  Now  you  will  readily  comprehend,  gentlemen,  how  com- 
plex  and  variable  may  appear  the  symptomatic  group  which  is 
observable  under  thèse  différent  combinations.  For,  in  fact,  there 
is  probably  not  one  chronic  elementary  lésion  of  the  cord  which  is 
not  susceptible,  at  a  given  period  of  its  évolution,  of  reacting  on 
the  anterior  grey  substance,  and  causing  atrophy  of  its  motor 
cells. 

To  speak  only  of  cases  in  which  an  anatomical  vérification  was 
made,  the  folio wing  is  a  statement  of  the  chief  forms  of  disease 
of  the  spinal  cord  which  may  give  rise  to  chronic  deuteropathic 
spinal  amyotrophy  : 

1°.  In  the  first  place,  I  will  mention  hypertrophie  spinal pachy- 
menhifjitis.  It  consists,  as  we  will  see,  in  an  inflammation  of  the 
méninges  which  especially  occupies  the  cervical  enlargement  of  the 
cord  and  which  unquestionably  corresponds  to  what  was  formerly 
called  hypertrophy  of  the  spinal  cord.  The  meningeal  lésion  is  pro- 
pagated  to  the  cord  itself  and,  simultaneously,  to  the  origin  of  the 
rachidian  nerves.  The  muscular  atrophy  of  the  upper  extremities 
is  developed  under  this  double  influence  ;  it  exhibits  itself  in  com- 
bination  with  peculiar  symptoms  which  are  due  to  the  meningeal 
lésion,  the  spinal  lésion,  and  the  lésion  of  the  peripheral  nerves. 

3°.  Next  cornes  sclerosis  of  the  posterior  radicular  zones,    the 
anatomical  substratum  of  progressive  locomotor  ataxia.^     The  sym- 
^  See  Lecture  I,  p.  11. 


166  DEUTEROPATHIO    SPINAL  AMTOTROPHIES. 

ptomatology  would  hère  be  composed  of  plienomena  connected 
with  consécutive  atrophy  of  the  anterior  cornua^ — slow  atropliy  of 
tlie  muscles, — and  of  tliose  wliicli  cliaracterise  sclerosis  of  tlie 
posterior  radicular  zones,  spécial  fulgurant  pains,  motor  incoordina- 
tion, &c. 

3°.  Différent  types  of  central  myelïtis,  spontaneous  or  traumatic, 
liaving  a  chronic  course^  should  be  included  in  this  enumeration  ; 
an  anatomical  lésion  wliich  is  commonly  designated  uuder  the 
name  of  hijilromijclïa  or  hijdfomyeliiis,  deserves  spécial  mention.' 

Some  authors  describe  this  spinal  altération  as  resulting  from  a 

1  This  spinal  lésion  bas  been  desiguated  by  Ollivier  (d'Angers)  under  the 
name  of  sjjrinfjomyelia  or  central  caviiy  in  the  spinal  cord  ('  Traité  des  maladies 
de  la  moelle  cpinière,'  3e  cdit,,  1837,  t.  i,  p.  202).  I  hâve  published  a  case  of 
cervical  spinal  myelitis  with  pachymeningitis,  remarkable  amongst  others  on 
account  of  the  présence  of  three  long  and  narrow  canals  which,  excavated  for 
the  most  part  in  the  substance  of  the  grey  matter,  traversed  the  cervical 
enlargement  throughout  its  whole  extent  iu  a  direction  parallel  to  the  long 
axis  of  the  cord.  One,  and  the  most  considérable,  of  thèse  canals  might  even 
be  followed  to  a  level  with  the  inferior  third  of  the  dorsal  région.  In  the 
greater  portion  of  its  course  it  occupied  the  posterior  grcy  cornu  of  the  left 
side,  or  rather  it  had  taken  the  place  of  this  grey  cornu,  the  several  éléments 
of  vphich  had  disappeared.  Of  the  two  other  canals,  smaller  in  size,  one 
occupied  a  position  immediately  bebind  the  posterior  commissure,  on  the 
médian  line,  so  as  to  involve  both  posterior  white  columns  at  the  same  time  ; 
■whilst  the  other  ran  partly  in  the  right  posterior  cornu,  and  partly  in  the 
posterior  colunin  of  the  right  side.  The  latter  canals  were  found  mostly  lilled 
by  an  aniorphous,  transparent,  finely-granulated  substance,  vpliich,  in  certain 
points,  had  bccome  disintegrated,  probably  owing  to  some  accident  in  prepar- 
ing  the  section,  and  had  lel't  in  its  place  more  or  less  extensive  lacunœ,  with 
more  or  less  irregular  borders.  This  same  fiuely-grauular  substance,  sliglitly 
condensed,  formed  the  parietes  of  the  foci  and,  without  any  well-marked  line 
of  démarcation,  became  continuons  with  the  adjacent  tissue  which  itself, 
for  some  distance,  presentcd  the  characters  of  granular  degeneration.  This 
case,  which  api)ears  in  a  memoir,  published  in  common  with  M.  Joffroy,  Ihen 
my  clinical  clerk  ('Archives  de  Physiologie,'  Mai,  Sept.,  Nov.,  1S69),  made  it 
already  seem  highly  probable  that  a  certain  number  of  cases  of  hydromyelia 
till  then  rather  commonly  referred  to  a  dilatation  of  the  central  canal,  might 
resuit  from  the  mclting  down  of  a  pathological  tissue,  developed  in  the  midst 
of  the  central  portions  of  the  spinal  cord.  The  reality  of  the  fact  seems  to 
me  to  hâve  been  placed  beyond  question  by  M.  Ilallopcau  in  an  intcresting 
work  presented  to  the  Société  de  Biologie  ('  Mémoires  de  la  Société  de  Biolo- 
gie,' 1869).  Quite  recently,  Dr.  Th.  Simon  (of  Hamburgh)  bas  colleeted  a 
large  number  of  cases  ('  xircli.  fiir  Psychiatrie  und  Nervenkrankeit,'  v  Bd.,  i 
Ileft,  Berlin,  1874,  p.  120,  et  serj.)  which  go  to  confirm,  as  regards  this 
subject,  the  conclusions  drawn  in  the  French  works. 


CAUSATIVE  SPINAL  AFFECTIONS.  167 

dilatation  of  tlie  central  canal  of  tlie  spinal  cord.  It  is  certain  tliat 
in  the  majority  of  cases,  we  hâve  to  deal  witli  cliannelled  foci,  con- 
sécutive on  clironic  central  myelitis.  However  it  be,  the  grey  sub- 
stance of  the  anterior  cornua  may,  in  similar  circumstances,  be 
involved  to  sucli  an  extent,  that  the  motor  nerve  cells  undergo 
more  or  less  grave  altérations  and,  owing  to  this  fact,  muscular 
atrophy,  having  a  progressive  course,  will  take  its  place  in  the  sym- 
ptomatology  of  the  disease.^ 

4°  There  also  exist  several  examples  of  inir a- spinal  tumours 
(gliomata  or  sarcomata),  which,  developed  in  the  centre  of  the  grey 
substance,  in  the  cervical  région,  hâve  formed  the  starting-point  of 
symptoms  of  progressive  amyotrophy.^ 

^.  We  should  also  mention  disseminated  sclerosis.  Generally 
speaking,  in  ordinary  cases  of  this  disease,  the  grey  substance  is 
not  gravely  affected  ;  that,  however,  may  possibly  occur,  and  then 
progressive  amyotrophies  w^ould  présent  themselves,  in  addition  to 
the  already  very  varied  symptoms  of  multilocular  induration  of  the 
nerve  centres. 

6°.  But  the  pathological  form  which  I  wish  particularly  to  notice, 
araongst  thèse  chronic  deuteropathic  spinal  amyotrophies,  is  one 
which  is  characterised  anatomically  by  a  sclerosis  wliïch  sijmmetri- 
cally  affecis  the  latéral  columns  of  tlie  spinal  cord  throughout  its 
whole  length.  This  fasciculated  sclerosis  may  even  be  followed,  as 
we  shall  show,  into  the  bulbus  and  the  protuberantia. 

Symmetrical  latéral  fasciculated  sclerosis  may  be  met  with, 
separately,  irrespective  of  any  lésion  of  the  grey  axis.  But  very 
frequently  it  reacts  upon  the  anterior  cornua  of  the  grey  substance, 
and  more  particularly  on  the  nerve-cells  of  the  région,  in  conséquence 
of  which  amyotrophic  symptoms  are  superadded  to  those  which 
dépend  on  latéral  sclerosis. 

In  ail  the  cases  comprised  within  this  enumeration  the  invasion 
of  the  anterior  grey  substance,  as  we  hâve  pointed  ont,  is  constantly 
a  consécutive  phenomenon.  It  is  possible  that  the  inverse  com- 
bination  may  supervene,  namely,  that  a  lésion,  primarily  developed 

*  See,  amongst  others,  the  case  of  H.  O.  Schuppel,  "  Ueber  ïïydromyelus," 
in  '  Arcbiv  der  Heilkunde,'  Leipzig,  1865,  p.  289. 

"  O.  Schuppel,  "Das  gliom  und  gliomyxoni  des  E,ûckeumarkes,"  in  'Arch. 
der  Heilkunde,'  p.  127,  1867  ;  J.  Grimm,  "  Atrophia  inusculorum  progressiva, 
tumor  carcinomatosus  intumescentiœ  spinalis,"  in  '  Virchow's  Archiv,'  4  faeg, 
8Bd. 


168  INDIVIDUAL  ATROPHi'  OP  MUSCLES. 

in  the  grey  centre,  may  subsequently  invade  the  white  columns  ; 
but  I  do  not  believe  that,  up  to  the  présent,  this  combination  lias 
been  regularly  observed. 

II. 

Gentlemen,  as  I  stated  to  you  at  the  outset,  it  is,  first  of  ail, 
protopatliïc  spinal  progressive  amyotrophj,  defined  as  you  hâve  just 
heard,  which  we  are  about  to  study  in  this  lecture.  "When  this 
comparatively  simple  type  is  known  to  you  it  will  be  more  easy  for 
you  to  penetrate  into  the  history  of  the  deuteropathic  spinal  amyo- 
trôphies,  which  is  still  rather  a  tangled  one. 

We  will  endeavour  to  separate  the  description  of  proiopatJiie 
muscular  atrophy,  as  much  as  possible,  from  ail  the  foreign  éléments 
which  encumber  it  in  the  pages  of  most  authors.  In  doing  so,  we 
shall  foUow  the  example  of  Dr.  Duchenne  (de  Boulogne),  who  has 
long  since  begun  this  work  of  purification,  taking  up  his  position 
at  a  clinical  standing  point  especially.  The  signais,  established  by 
that  author  along  the  road  which  he  has  already  travelled,  shall  more 
than  once  serve  to  guide  us  in  accomplishing  the  task  which  we  are 
about  to  undertake.^ 

A.  We  will  begin  our  account  by  taking  first  the  clinical  side  ; 
after  which  we  will  descend  into  the  détails  of  the  anatomical  lésion, 
and,  finally,  by  way  of  conclusion,  we  will  offer  some  considérations 
in  relation  to  the  pathological  physiology  of  the  affection. 

a.  The  first  feature  to  notice  in  the  symptomatology  of  pro- 
gressive muscular  atrophy,  after  the  insidious  invasion,  without 
forewarning  symptoms,  or  with  long  unnoticed  prodromes,  so  to 
say,  is  what  may  be  called  the  individual  a^ro^y^y  which  the  affected 
muscles  undergo  ;  in  other  words,  one  or  several  muscles  of  a 
member  may  hâve  suffered  a  very  remarkable  decrease  of  volume, 
whilst  the  adjoining  muscles  hâve  preserved  their  normal 
prominence. 

This  first  feature  is  in  some  sort  characteristic  ;  it  is,  writes  M. 
Duchenne  (de  Boulogne),  "the  faciès  of  tbe  disease.^^  Let  us 
exactly  realise  the  matter  by  appealing  to  a  concrète  example.  Let 
us  suppose  a  case,  one  very  common  in  this  order,  in  which  the 
disease  shall  as  yet  hâve  invaded  but  a  certain  number  of  muscles 
in  an  upper  extremity.     Ail  the  muscles  of  the  hand  and  of  the 

'  See  'Traite  de  l'Electrothérapie  localisée." — Ch.  v. 


FUNCTIONAL  DISOEDEKS.  169 

forearm  shall  hâve  undergone  intense  atrophy,  I  will  suppose,  witli 
the  exception  of  perhaps  one  only — the  supinator  longus,  for  in- 
stance. On  the  other  hand,  the  muscles  of  the  arm  and  of  the 
shoulder  will  be  untouched  and  présent  their  normal  volume,  so  as 
to  form  a  striking  contrast  with  the  intense  atrophy  of  the  hand  and 
forearm. 

Let  us  take  another  and  a  rarer  instance.  In  this  case  it  shall 
be  the  thoracic  muscles  which  hâve  been  first  affected.  The  pectorals 
will  be  intensely  wasted,  and,  consequently,  the  chest  will  hâve 
suffered  extrême  emaciation,  whilst  the  upper  extremities,  having^ 
been  entirely  spared,  will  présent  a  comparatively  considérable 
prominence.  This  mode  of  invasion  of  the  atrophy,  which  advances 
to  a  certain  extent  muscle  hj  muscle,  furnishes  an  important  cha- 
racter,  because  it  is  met  with  again,  to  the  same  degree,  in  deutero- 
pathic  amyotrophies. 

h.  The  functlonal  disorders  represented  by  the  muscle,  when 
atrophying,  sliould  arrest  our  attention.  With  decrease  in  volume 
is  connected  a  certain  degree  of  enfeehlement  of  the  movements 
executed  by  the  muscle,  and  we  may  say  that  thèse  two  phenomena 
advance  in  parallel  lines.  In  other  words,  the  fewer  muscular 
fibres  there  are  in  a  muscle,  or  the  more  its  fibres  are  atrophied, 
the  greater  will  be  the  debility,  and  this  appears  hardly  to  dépend 
on  anything  else  than  on  the  decrease  in  the  number,  or  more  or 
less  marked  atrophy  of  the  muscular  bundles. 

This  fact  contrasts  with  what  we  know  in  relation  to  paralijsls 
properly  so  called,  or  by  defauU  ofnerve  aciion.  Take,  for  instance, 
a  paralysis  of  the  lower  extremities  caused  by  compression,  bearing 
on  a  limited  point  of  the  spinal  cord  in  the  dorsal  région  ;  the 
motor  inertia  occasioned  in  the  inferior  limbs  by  the  suppression  of 
cérébral  action  may  be  complète,  absolute,  and  yet  the  muscles,  in  such 
a  case,  will  not  suffer  at  ail  in  their  nutrition,  or  only  suffer  in  the 
long  run,  owing  to  the  fact  of  their  prolonged  inaction. 

In  deuteropathic  spinal  amyotrophies,  on  account  of  the  habituai 
combination  of  a  lésion  of  the  white  colamns  with  the  lésion  of  the 
grey  substance,  it  is  the  rule  that  a  more  or  less  marked  degree  of 
paralysis,  by  suppression  of  nerve  action,  shall  be  superadded  to 
the  effects  of  the  amyotrophy,  which  does  not  take  place,  at  least 
not  to  the  same  extent,  in  protopathic  amyotrophy  where  the  grey 
substance  is  alone  affected. 

c.  Another  fact  worthy   of  notice  is  the  following  :  the  muscle. 


170  FIBRILLARY  QUIVERING.       DEFORMATIONS. 

cven  when  it  has  reached  an  advanced  degree  of  atropliy,  préserves 
its  normal  faradaic  contractility.  The  diminution  or  abolition  of 
this  contractility  only  shows  itself  in  the  ultimate  phases,  when  the 
atrophy  has  attained  its  climax.  This  is  a  character  which  contrasts 
considerably  with  what  we  know  of  acute  spinal  arayotrophies 
where,  from  an  early  date,  and  even  before  the  mass  of  the  muscle 
has  betrayed  by  its  change  a  noticeable  altération,  the  faradaic 
contractility  has  already  been  remarkably  modified. 

d.  It  is  important  not  to  forget,  in  the  description,  ûiq  fibrillari/ 
quivering.  This  quivering  is  spontaneously  produced,  but  we  may 
often  provoke  its  appearance  by  means  of  a  slight  shock  given  to 
the  muscle.  It  consists,  allow  me  to  remind  you,  in  this,  namely, 
that  the  skin  covering  the  affected  muscle,  appears  suddenly 
raised  by  very  fine  little  strings,  which  stretch  in  the  direction  of 
the  princijjal  muscular  fascicles.  Sometimes  quite  partial  and 
localised,  they  are  occasionally  energetic  enough  to  move  the  finger, 
or  even  the  hand.  Thèse  fibrillary  moveinents  do  not  peculiarly 
belong  to  the  protopathic  form.  I  will  add,  also,  that  they  are  to 
be  met  wàth,  bcyond  the  limits  of  progressive  muscular  atrophy,  in 
hcalthy  subjects.  They  occasionally  constitute,  in  this  case,  one 
of  the  sym])toms  of  a  particular  form  of  hypochondria  which, 
be  it  said  in  passing,  is  somewhat  fréquent  amongst  médical 
students. 

e.  I  ought  to  point  ont,  but  only  in  order  to  eliminate  them  from 
the  picture,  somc  other  symptoms  which  in  my  opinion,  bave  been 
wrongly  included  in  the  description  of  common  muscular  atrophy. 
The  spontaneous  (continued  and  neuralgic)  pains,  the  fulgurant 
paroxysmal  pains,  raentioned  by  soine  authors,  belong  to  the  deuter- 
opathic  forms  (posterior  sclerosis,  latéral  symmetrical  sclerosis, 
pachymeniugitis) . 

I  will  say  as  much  of  the  cutaneous  anœsthesia  and  hyperses- 
thesia.  They  are  foreign  to  simple  atrophy.  The  same  statement 
holds  good,  according  to  my  expérience,  with  respect  to  the  pains, 
provoked  by  pressure,  which  betray  an  exaltation  of  the  sensibility 
of  the  muscular  masses. 

/.  Lastly,  I  should  include  in  the  symptomatology  of  protopathic 
spinal  atrophy  the  déformations  or  rather  the  déviations  which, 
necessarily,  resuit  from  the  weakening  of  the  wasted  muscles  and 
the  prédominance  which  the  antagonist  muscles  consequently 
assume.      Thus  is  produced,  to  mention  merely  the  hands,  the 


MODES  OF  INVASION.  171 

various  déformations  whicli  are  kuown  by  the  naine  of  "griffes," 
(claws  or  talons) . 

In  short,  we  hâve  hère  paralftic  déviations  which  must  not  be 
confounded  with  déformations  due  to  contracture^  which  show 
themselves  in  certain  deuteropathic  forms  and  there  perform  an 
interesting  part  as  raay  be  seen,  to  take  one  instance,  in  latéral 
amyotrophic  sclerosis. 

B.  After  this  enunieration  of  the  syraptoms  which  are  observed 
in  each  of  the  affected  muscles,  considered  separately,  we  should 
fix  our  attention  on  some  characters  drawn  from  the  mode  of  pro- 
gression and  distribution  which  is  presentcd  by  the  muscular  lésions 
in  their  successive  invasions.  In  this  order,  we  hâve  to  point  eut 
a  certain  number  of  phenomena  of  incontestable  utility  for  noso- 
graphic  differentiation  : 

1°.  In  the  immense  majority  of  cases,  protopathic  spinal  pro- 
gressive amyotrophy  begins  by  one  of  the  iipper  extremities;  it 
commences  with  the  haud  and  ascends  the  forearm,  the  arm,  the 
shoulder,  gaining  then  tlie  trunk.  As  a  rule,  a  fact  which  I  ask 
you  to  remark  particularly  as  we  shall  utilise  it  presently,  it  only 
extends  to  the  inferior  extremities  when  the  disease  lias  reached  its 
ultiraate  limits.  I  hâve  oft-times  shown  you,  in  this  asylum,  pa- 
tients stricken  with  protopathic  muscular  atro])hy  of  long  standing, 
whose  upper  extremities,  and  thorax  as  well,  were  reduced  to  a 
skeleton  ;  whilst  the  lower  extremities,  either  not  at  ail  or  but  little 
afifected,  permitted  the  patient  to  stand  and  to  walk,  nearly  as  in 
the  normal  condition. 

3°.  Primary  invasion  of  the  trunk  is  far  more  rare.  Dr.  Duchenne 
has  noted  it  in  a  dozeri  of  cases  only  :  the  upper  extremities  are 
hère  attacked  subsequently. 

3°.  Finally,  it  is  proper  to  mention,  as  a  most  exceptional  mode 
of  invasion,  one  which  is  very  rare  in  common  atrophy  (the  con- 
verse will  be  seen  in  some  deuteropathic  atrophies),  that  mode  in 
which  the  muscles  of  the  inferior  extremities  are  taken  before  ail 
the  others.  Dr.  Duchenne  says  that  he  has  not  observed  this 
mode  of  invasion  except  twice  in  159  cases.  Mr.  Hammond,i 
indeed,  in  a  récent  treatise  déclares  that  he  has  seen  it  8  times 
in  29  ;  but,  if  I  am  to  judge  by  one  of  the  examples  which  he 
relates,  the  cases  which  hâve  served  for  thèse  statistics  deviate  sin- 

'  W.  A.  Hamniond,  '  A  Treatise  on  Diseases  of  the  Nervous  System,'  p. 
666,  fig.  31,  New  York. 


172  PROGEESS.       ETIOLOGY. 

gularly  from  the  classic  type.  The  case  to  which  I  refer  relates  ta 
a  man  who,  after  having  experienced  some  disorder  of  vision^  and 
suffered,  for  a  long  time,  from  formications  as  well  as  pains  ("elec- 
tric  pains  ")  in  the  lower  extremities,  presented  a  growing  pro- 
gressive atrophy  of  the  muscles  of  thèse  members,  •which  had 
wasted  them  to  a  considérable  extent.  This  atrophy  of  the  mus- 
cular  masses,  great  as  it  was,  did  not  absolutely  hinder  the 
patient  from  standing  and  walking.  I  cannot  refrain  from  seeing, 
in  this  case,  an  example  of  locomotor  ataxia  ;  it  is  known  that, 
in  this  affection,  progressive  atrophy  of  the  members,  stricken 
with  motor  incoordination,  is  not  a  very  rare  complication. 

c.  It  may  be  asserted  that,  under  ordinary  conditions^  the 
course  of  the  disease  is  a  very  slow  one  ;  it  is  the  exception  when 
prematurely  invading  the  muscles  which  serve  for  the  work  of 
respiration  (intercostals  or  diaphragm),  or  attacking  the  bulbar 
nerves  (a  combination  which  shall  be  specially  discussed),  the  dis- 
ease runs  to  a  close  in  from  two  to  five  years.  Usually,  I  repeat 
true  atrophy  lasts  eight  or  ten  years,  in  its  partial  form,  and  even 
eighteen  or  twenty  years  when  it  has  been  long  generalised  over  ail 
the  muscles. 

D.  One  Word  now  with  respect  to  its  etiologi/.  What  has  been 
written  in  référence  to  consanguinity  or  heredity,  considered  as  au 
élément  in  etiology,  seems  to  me,  judging  from  a  critical  examina- 
tion  of  the  texts,  to  apply  to  protopathic  spinal  amyotrophy.  The 
latter,  I  should  add,  is  rarely  affiliated  to  occasional  causes, 

The  sjmial  amyotrojplàes  which  are  called  rheumatic,  because  they 
appear  to  hâve  been  caused  by  the  influence  of  cold  are,  if  I  am  not 
mistaken,  referable  to  chronic  mjelitis,  pachymeningitis,  or  latéral 
sclerosis. 

Those  which  supervene  in  conséquence  of  a  traumatic  cause,  sucli 
as  a  blow  on  the  back,  as  in  a  case  related  by  Dr.  Gull,^  or  from 
carrying  too  heavy  a  baie  of  cotton,  as  in  the  case  of  Dr.  Eobert's 
patient,  &c,_,  are  probably  also  connected  with  myelitis." 

'  W.  Gull,  "  Progressive  Atrophy  of  the  Muscles  of  the  Trunk  and  Upper 
Extremities,  after  a  blow  on  the  neck  with  the  fist,"  in  '  Guy's  Hosp.  Reports,' 
1858,  p.  195. 

2  W.  Roberts,  Art.  "  Wasting  Palsy,"  in  '  Reynolds'  System  of  Mediciue, 
p.  168.  Other  examples  of  progressive  atrophy  of  muscles,  supervening  on 
injuries,  arementioned  byBergraann,  'St. Peterbourger Med.-Zeitsch.,'  p.  116, 
1 864.  Thudichum  und  Lockbart  Clarke  ('  Beale's  Archives  of  Médecine,'  1 863) . 


PATHOLOGICAL  ANATOIIY.  173 

But,  so  far  as  primary  atrophy  is  concernée!,  tlie  etiologic  rôle  of 
hereditary  transmission  appears  to  be  a  very  important  one.  It  has 
been  noticed  by  ail  authors,  and  recently  H.  Naûnyn,  Professor 
at  Ivonigsberg,  narrated  the  history  of  a  family  in  wliich  tlie 
transmission  of  the  disease  could  be  traced  througli  five  généra- 
tions .1 

III. 

In  an  account,  wliich  is  chiefly  concerned  witli  rendering  évident 
some  fundamental  nosographical  characters,  enough  lias  been  said, 
gentlemen,  with  référence  to  the  clinical  aspect  of  tlie  question. 
We  hâve  coUected,  on  our  way,  materials  the  utility  of  which  may 
not  strike  the  eye  at  the  first  glance,  but  this  will  appear  in  its 
fulness  when,  in  a  few  minutes,  we  shall  hâve  to  show  how  deutero- 
pathic  amyotrophies  are  distinguishable  from  protopathic  amyo- 
trophy,  not  only  anatomically,  but  also  by  the  entire  array  of  other 
pathological  characters. 

At  présent,  it  is  proper  to  make  you  acquainted  with  what  is 
known  as  regards  the  patliological  anatomy  of  protopathic  spinal 
progressive  amyotrophy. 

1°.  We  will  commence  by  what  relates  to  the  cord.  The  lésion 
in  question,  necessarily  affects  the  great  motor  cells. 

The  neuroglia  itself  may  also  be  attacked,  but,  in  suc  h  circum- 
stances,  the  altération  remains  systematically  circumscribed  within 
the  grey  anterior  cornua  ;  the  white  columns  are  completely 
respected. 

The  lésion  of  the  neuroglia  is  in  its  nature  an  inflammation  ; 
îience  the  vessels  of  the  grey  substance  are  larger  than  usual  and 
their  walls  are  thickened.  The  cellular  éléments  of  the  connective 
matrix  présent  évident  traces  of  a  process  of  prolifération.  Granular 
bodies,  in  varying  number,  are  met  with  in  po'eparations  made  in 
the  fresh  state.  When  thèse  altérations  of  the  neuroglia  are  very 
marked  the  grey  cornu  may  show  a  diminution  in  ail  its  diameters 
(fig.  12,  p.  174). 

With  respect  to  the  nerve-cells,  they  exhibit  sometimes  the 
characteristics  of  pigmentary  atrophy  (fig.  11,  c,  d,  p.  156),  and 
sometimes  those  of  scierons  atrophy  (fig.  11,  e,  p.  156). 

In  short,   we  find  hère,   in  a  chronic  form,  altérations  whijch 

1  '  Berliu  Klin.  Woclienschr.,'  No.  42,  iS7.q. 


174 


LESIONS  OP  SPINAL  OOED. 


we  hâve  described  in  connection  witli  infantile  paralysis  ;  and  there 
is  reason  to  suppose  tliat,  as  in  the  latter  case,  the  limiting  of  the 
altérations  to  the  anterior  cornua  of  the  grej  substance  is  accounted 
for  by  the  circunistance  that  the  ganglionic  éléments  are  the  primary 
seat  of  the  aflection^. 

Considered     iu    référence    to    its    anatomical    charaeters^    the 


JFiG.  12. — Section  of  the  spinal  cord  in  the  cervical  région  in  a  case  of  pro- 
topathic  muscular  atrophy.  a.  Left  anterior  cornu  (the  nerve  cells  hâve 
persisted,  but  présent  signs  of  qualitative  change).  B.  Right  anterior 
cornu  (almost  complète  atrophy  of  the  nerve  cells,  ouly  one  sniall  ganglionic 
nucleus  {p)  rcmains). 

form  of  progressive  muscular  atrophy  under  discussion  might 
be  designated  by  the  name  of  chronic  parenchymatous  tepliro- 
myelitis. 

The  observations  on  which  the  foregoing  anatomo-pathological 
description  is  based,  are  not  numerous  as  yet.  There  are  only  six 
or  seven,  we  think,  in  existence.  Amongst  thèse  we  would  mention 
a  case  described  by  Dr.  Lockhart  Clarke,  another  by  M.  Duménil, 


^  See  Lecture  IX,  p.  139,  and  Lecture  X,  p.  15;^ 


LESIONS  OP  SPINAL  COED.  175 

of  Rouen,^  a  very  important  case  by  M.  Hayem  f  tliree  cases^  noted 

^  Unfortunately,  iu  tlie  cases  of  Lockhart  Clarke  and  Duménil,  the  state  of 
tlie  wLite  spinal  substance  is  not  exjtlicitly  indicated. 

^  Hayem,  "  Note  sur  un  cas  d'atrophie  musculaire  progressive,  avec  lésions 
de  la  moelle,"  '  Archives  de  Physiologie,'  1869,  p.  79. 

^  The  foUowing  is  a  summary  of  two  of  thèse  observations  which  will  soou 
be  published,  in  extenso,  in  the  '  Archives  de  Physiologie.'  The  third  has 
already  appeared  in  its  pages  (1870,  p.  247).  I  bave  giveu  it  as  an  example 
of  glosso-laryngeal  paralysis. 

Observation  I,  noted  by  M.  Gombault. — A  Duc — ,  scliool-teacher,  aged 
fifty-six,  was  received  into  Salpêtrière  Hospital  24tli  June,  1872  ;  died  26tk 
Sept.,  same  year.  She  suffered  greatly  from  cold  and  fatigue  during  the  siège. 
Attacked  in  April,  1871,  by  progressive  weakeningof  the  left  upper  extremity. 
Difficulty  of  speech  occurring  at  nearly  same  epoch.  No  pains  or  contractures 
in  the  limbs.  Présent  state  in  July,  1872  :  well-marked  paralytic  torticollis, 
with  compensation  curves  in  the  rcmainder  of  tiie  spinal  column.  Symptoms 
of  labio-glosso-laryngeal  paralysis,  with  manifest  atrophy  of  the  tongue. 
Well-marked  difficulty  in  swallowing.  The  two  'upj)er  extremities,  the  left 
especialiy,  are  hanging,  inert,  without  contracture.  Their  muscular  niass  is 
wasted  nearly  uniformly  throughout.  Fibrillary  conti'actious  very  évident. 
Faradaic  contractility  not  changed.  In  the  hand,  almost  complète  disappear- 
ance  of  the  thenar  and  hypothcnar  eniinences.  There  is  no  "griffe"  deformity. 
Loioer  extremities  not  atrophied  ;  standing  and  walking  werc  possible  up  to  the 
last  moment.     Deatli  rapid,  owing  to  lobular  pneumonia. 

State  of  spinal  conl  on  autopu'i/. — Cervical  enlargement  ;  in  the  fresh  state, 
grauular  bodies  exist  iu  abuudance  within  the  area  of  the  anterior  cornua 
exclusively.  The  motor  nerve-cells  there  présent  ail  possible  degrees  of 
pigmentary  degeneration.  On  hardened  sections  same  altération  of  nerve-cells 
is  found.  Many  of  them  are  only  represented  by  a  globular  mass  of  pigment. 
Numerous  islets  of  granular  disintegration  within  the  area  of  the  anterior 
cornua.  The  white  columns  and  the  latéral  columns  in  particular  do  not  présent 
the  least  trace  of  alteratio7i. 

ObsefcVation  II,  noted  by  M.  Pierret. — The  patient  C —  was  received 
into  the  Salpêtrière  on  the  i8th  Feb.,  1850  ;  died  April  I4tb,  1874,  aged  fifty- 
five.  Affection  began,  at  the  âge  of  twenty-six,  by  the  right  upper  extremity. 
The  lower  extremities  were  not  affected  so  as  to  make  walking  impossible, 
until  five  or  six  ycars  ago.  No  contractures,  no  disturbancc  of  sensibility, 
very  wcU-marked  fibrillary  contractions.  Latterly  ail  movements  of  the 
limbs  hâve  become  nearly  impossible.  The  wasting  of  the  muscles  is 
especialiy  noticeable  in  the  right  thoracic  extremity  (hand,  shoulder,  forearm). 
Death  supervened,  iu  conséquence  of  pulmonary  tuberculisation  rapidly 
evolved. 

Examination  of  sections  of  the  hardened  spinal  cord. — Throughout  the  whole 
extent  of  the  cord,  but  especialiy  in  the  cervical  région,  a  great  number  of 
nerve  cells  of  the  anterior  cornua  hâve  disappeared  without  leaving  any  trace  ; 
those  which  resist  are  very  small  and  présent  the  most  advanced  degrees  of 
pigmentary  degeneration.     On  a  level  with  the  fourth  cervical  pair  of  nerves. 


176  LESIONS  OF  ANTERIOE  NERVE-EOOTS,  ETC. 

at  the  Salpêtrière  in  my  wards  ;  and,  lastly,  a  case  in  every  respect 
very  regular,  was  recently  observed  in  the  wards  of  Professer  Vul- 
pian,  by  Dr.  Troisier.^ 

This  small  array  of  cases  forms,  nevertheless,  a  solid  whole.  AU 
contradictory  observations,  that  is  to  say,  those  in  which  progressive 
atrophy,  according  to  the  Duchenne-Aran  clinical  type,  is  supposed 
to  show  itself  without  the  accompaniment  of  the  spinal  lésions 
described,  are,  I  believe  I  hâve  ascertained,  defective  either  clinically 
or  anatomically.  With  respect  to  the  latter  point,  gentlemen,  I 
would  hâve  you  remark  that  the  spinal  lésion  of  protopathic  pro- 
gressive arayotrophy,  as  well  as  that  of  infantile  paralysis,  cannot 
be  identified  with  certainty  except  when  properly  prepared  sections 
hâve  been  subjected  to  examination.  Ail  investigations  which  hâve 
not  been  carried  ont,  in  accordance  with  this  method,  and  under 
conditions  favourable  to  success,  should,  in  relation  to  this  matter, 
be  considered  nuU  and  void.^ 

the  right  anterior  cornua  lias  suffered  réduction  in  every  diameter  ;  its  neuroglia 
is  evidently  sclerosed  ;  the  motor  nerve-cells  hâve  disappeared  with  the  excep- 
tion of  only  one  little  group  (fig.  ii,  b).  The  anterior  roots  emanating  from 
the  cervical  région  are  wasted  ;  some  nerve  tubes  are  found  there  exhibiting 
granular  fatty  degeneration. 

*  The  observation  of  M.  Troisier  will  be  published  shortly  in  the  '  Archives 
de  Physiologie.' 

2  Dr.  Bamberger  has  published  ('Wiener  Mediz.  Presse,'  Nos.  27,  28,  Juli, 
1869,  and  '  Centralblatt,'  Oct.,  No.  46,  1869)  two  cases  of  progressive  muscular 
atrophy,  in  vrliich  the  autopsy,  made  by  Prof.  Recklinghausen,  did  not,  it  is 
stated,  permit  the  récognition  of  any  lésion  of  the  spinal  cord.  Unfortunately, 
the  account  of  the  autopsy,  so  far  as  the  nervous  System  is  concerned,  is  not 
accompanied  by  circumstantial  détails,  in  thèse  cases.  It  is  not,  for  instance, 
mentioned  if  the  microscopic  examination  was  made  with  hardened  sections, 
which  hère  is  absolutely  indispensable,  and  the  condition  of  the  cells  of  the 
anterior  cornua  is  not  specially  stated.  We  consider  it  necessary  once  more 
to  recall  the  fact  that  the  spinal  cord  may  appear  quite  healthy,  to  the  naked 
eye,  at  a  time  'vi'hen  the  nerve-cells  of  the  anterior  cornua  hâve,  nevertheless, 
undergone  the  niost  complète  altérations,  We  would  add  that,  in  such  a  case, 
microscopical  examination  itself  may  not  furnish  any  decided  resuit  if  it  be 
made  with  sections  not  hardened. 

The  preceding  remarks  apply  in  every  point  to  ail  the  cases,  without  excep- 
tion, which  Herr  Friedreich  has  arrayed  in  his  récent  work  ('  Ueber  Progres- 
sive Muskelatrophies,'  Berlin,  1873)  against  the  nerve  theory  of  progressive 
amyotrophy  (Obs.  i,  ii,  iv,  x,  ai>d  xvii).  I  speak  of  the  observations  collected 
by  the  author  himself  ;  ail  date  from  a  period  (from  1858— 1867)  when  the 
rôle  of  the  altération  of  the  nerve-cells,  tliemselves,  had  not  been  yet  clucidated, 
and  nowhere  is  the  auatomical  condition  of  thèse  cells  explicitly  mentioned. 


LESIONS    OF  ANTEEIOR   NEEVE-EOOTS.  177 

2°.  The  anterior  nerre-roots  and  i\\Q  peripJieral  nerves  are  S/ffected 
consecutively  to  the  lésion  of  the  grey  substance.  I  would  remind 
you,  in  référence  to  this,  that  Cruveilhier  considered  the  atrophy  of 
the  anterior  nerve-roots  as  the  anatomical  character  of  that  form  of 
muscular  atrophy  the  history  of  which  he  contributed  to  elucidate^  and 
that  he,  as  it  were,  foresaw  that  this  atrophy  would  some  day  be 
correlated  to  a  lésion  of  the  grey  substance.^ 

It  is  important  to  note  that  the  atrophy  of  the  anterior  roots 
cannot  be  a  destructive  atrophy  to  the  same  extent  as  in  infantile 
paralysis.  Undoubtedly,  in  the  roots  emanating  from  région? 
where  the  grey  substance  is  most  seriously  altered,  we  usually  find 
a  certain  number  of  nerve-tubes  void  of  medullary  matter,  or  in 
which  this  substance  is  affected,  in  varying  degrees,  with  fatty 
granular  degeneration.  But  the  greater  portion  of  thèse  tubes 
may,  as  I  hâve  again  verified  in  a  récent  case,  be  preserved  intact,  or, 
at  most,  présent  no  other  altérations  than  those  of  simple  atrophy. 
This  comparative  integrity  of  a  very  great  number  of  nerve-tubes 
of  the  anterior  roots  is  seen  even  when  thèse  ofiFer  to  the  naked  eye 
a  very  slender  appearance  and  a  slightly  greyish  tint. 

The  mechanism  by  which  the  spinal  altération  reacts  upon  the 
muscles  in  progressive  amyotrophy  seems,  therefore,  to  difFer,  in 
some  respects,  from  that  which  is  observed  in  infantile  paralysis. 
In  the  latter,  a  certain  number  of  nerve-tubes  take  on  the  same 
changes  as  a  divided  nerve.  In  muscular  atrophy,  only  a  few  nerves 
expérience  this  fate,  and  even  then  the  destruction  is  effected  little 
by  little,  progressively,  and  is  only  definitely  accomplished  in  the 
last  stages  of  the  morbid  process.  The  other  tubes  of  the  nerve- 
root  préserve  the  integrity  of  their  constitution,  at  least  in  ail 
essential  particulars. 

I  would  also  point  eut  that  several  of  the  cases,  grouped  by  Herr  rriedreicli 
under  the  same  heading,  do  not  at  ail  deserve  the  name  of  progressive  muscular 
atrophy,  even  employing  this  terra  in  its  widest  and  vaguest  sensé.  Thus 
Cases  I  and  II  can  hardly  be  taken  as  other  than  examples  of  infantile 
spinal  paralysis  ;  and  Case  VI,  remarkable  by  its  fébrile  commencement  and 
the  rapid  course  of  the  disease,  seems  to  me  to  belong  naturally  to  the  type 
created  by  Duchenne,  under  the  désignation  of  spinal  paralysis  of  the  adult. 
Such  a  laisser  aller  in  the  niatter  of  nosographic  differeutiation,  especially  in 
a  question  of  itself  rather  obscure,  is  to  say  the  least  regretable  and  can 
only  continue  the  confusion. 

^  Cruveilhier,  'Bulletin  de  l'Académie  de  Médecine,'   1853;  id.,  'Sur  la 
Paralysie  Musculaire  Atrophique,'  5e  série,  t.  vii,  Janvier,  1856. 
VOL,  II.  12 


178  MUSCULAR  LESIONS. 

What,  tlien,  is  the  mode  according  to  wliicli^  in  progressive 
amyotrophy,  the  muscular  lésion  is  effected  in  conséquence  of  tlie 
spinal  lésion  ?  With  respect  to  this  question,  I  cannot  well  see  any 
but  one  hypothesis  that  may  be  proposed_,  namely,  tliat  tlie  irrita- 
tive  action,  occupying  the  cells,  is  transmitted  by  means  of  the 
nerve-roots  and  centrifugal  nerves  to  the  muscular  bundles  which, 
under  this  influence,  sulfer  trophic  lésion.  The  atrophy  is  hère  the 
primary  phenomenon  :  it  is  not  at  first  accompanied  by  paralysis 
from  interruption  of  nervous  influx,  because  the  latter  may  still 
proceed  for  a  long  while  by  the  channel  of  the  emaci'ated,  but  not 
annihilated,  nerve-tubes. 

3°.  It  remains  for  me  now,  finally,  to  show  you  in  what  consist 
thèse  muscular  lestons,  whicb  thus  supervene  in  conséquence  of  the 
lésions  of  the  spinal  centre.  I  shall  not  need  to  dwell  long  on  this 
point,  for  many  are  the  analogies  which  connect  the  muscular 
lésions  of  protopathic  spinal  amyotrophic  with  those  of  infantile 
paralysis. 

This  question  of  pathological  histology  bas  been,  in  its  time,  the 
object  of  many  controversies,  which  are  very  interesting  to  examine 
from  a  critical  and  historical  point  of  view,  but  which  hâve  other- 
wise  lost  most  of  their  value.  I  will  only  remind  you  that  the  fatty- 
granular  degeneration  of  a  certain  number  of  fibres  is  the  fact  by 
which  the  first  observers — Mandl,  Galliet,  Lebert,  Cruveilhier, 
Aran,  and  Duchenne  —  were  especially  struck.  Taking  thèse 
observations  as  a  basis,  Dr.  Duchenne  believed  he  could  charac- 
terise  the  disease,  anatomically,  by  giving  it  the  name  oi progressive 
fatty  muscular  atrophy. 

Professor  Robin  then  interposed  in  the  debate,  and  he  rightly 
remarked  that  many  of  the  granulations  which  appear  on  the  fibres 
are  not  of  a  fatty  nature,  since  they  dissolve  in  acetic  acid,  whilst 
they  resist  the  action  of  ether.^ 

Next  came  Professor  Virchow,  who  protested  on  behalf  of  fatty 
degeneration,  and  even  went  beyond  the  opinions  that  had  been 
advanced,  pointing  out  the  fact,  which  is  quite  accurate,  that  the 
fat  does  not  originate  within  the  muscular  fibre  only,  but  that  it 
also  occasionally  invades  the  interstitial  connective  tissue  or  perï- 
nysmm.  It  is  easy  to-day  to  perceive  that,  in  this  debate,  the 
essential  fact  was  allowed  to  pass  nearly  unnoticed.     In  truth,  in 

'  Cil.  Robin,  '  Comptes  rendus  et  Mém.  de  la  Soc.  de  Biologie,'  1854, 
p.  201. 


I 


NATUEE    OP  LESIONS.  179 

progressive  spinal  amyotrophy,  as  in  infantile  amyotrophy,  granulo- 
proteic  degeneration,  as  well  as  granular-fatty  degeneration  of  the 
muscular  fibres,  is  only  an  accessory  phenomenon.  This  is  proved 
by  the  case  of  M.  Hayem,  and  by  the  multiplied  observations  which 
we  hâve  made  at  tlie  Salpêtrière. 

The  capital  fact,  in  this  matter,  is  a  simple  atrophy  of  the 
muscular  fibre,  with  rétention  of  the  cross  striation.  The  latter 
pefsists  to  the  ultimate  limits.  This  amyotrophy  is  usually  accom- 
panied,  on  some  points,  by  a  more  or  less  marked  prolifération  of 
the  cellular  éléments  of  the  sarcolemma.  In  a  certain  number  of 
muscular  fibres  this  multiplication  may  be  carried  far  enough  for  the 
new-formed  éléments  to  accumulate  within  the  sheath  of  the  sarco- 
lemma, so  as  to  distend  it  and  crowd  back  the  muscular  sub- 
stance. The  latter  then  becomes  segmented,  and  présents  the 
appearance  of  small  blocks,  which,  however,  préserve  their  striated 
markings  to  the  last  phases  of  the  altération. 

As  regards  the  newly-formed  cellular  éléments,  some  of  them 
take  on  the  development  of  cells  with  protoplasm,  but  this  is  rare. 
Most  of  them  become  abortive  in  their  évolution,  and  tend  to 
atrophy  ;  whilst,  at  the  same  time,  the  muscular  substance  goes  on 
dividing  and  subdividing,  and  sometimes  it  disappears  altogether, 
without  exhibiting  the  slightest  trace  of  granular  fatty  degenera- 
tion. AU  thèse  peculiarities  hâve  been  studied  with  care  in  the 
work  of  M.  Hayem. 

Finally,  the  perimysium  also,  to  a  certain  extent,  undergoes 
proliferative  action,  which  constitutes  a  sort  of  sketch  of  muscular 
cirrhosis.  In  its  turn,  interstitial  lipomatosis  may  intervene,  and 
even  proceed  to  luxuriant  lipomatosis.  The  latter  fact  deserves 
particular  mention,  because  fatty  deposit  may,  during  life,  render 
it  impossible  to  perçoive  the  existence  of  the  atrophy  of  the  mus- 
cular masses,  and  thus  mask  the  principal  symptom  of  the 
disease. 

Such,  gentlemen,  are  the  muscular  altérations  in  protopathic 
progressive  amyotrophy.  We  shall  shortly  see  that  they  do  not 
exclusively  belong  to  that  pathologie  form,  and  that  they  are  found 
with  the  same  characteristics  in  the  symptomatic  amyotrophies 
which  are  now  to  engage  our  attention. 


LECTURE  XII. 

DEUTEROPATHIC  SPINAL  AMYOTROPHIES.    LATERAL  AMYO- 
TROPHIC  SCLEROSIS. 

SuMMARY. — Deuteropatldc  spinal  aymjotropJiies.  Latéral  atnyotro- 
jpJiic  sclerosis  ;  localisation  of  the  spinal  lésion  in  the  latéral 
columns.  Causes  of  tJiis  localisation,  elncidated  hy  a  study  of 
the  development  of  the  sjnnal  cord.  Formation  of  latéral 
columns;  of  GolVs  columns  and  of  TërcJc's  columns. 

Latéral  sclerosis  consécutive  on  cérébral  lésion. 

Frimary  symmetrical  latéral  sclerosis.  Fathological  ana- 
tomy.  Appearance  and  topography  of  the  lésion  in  the  cord 
and  bulbîis.  Consécutive  lésions  of  the  grey  substance  {motor 
nerve-cells,  neuroglia),  in  the  cord  and  in  the  bulbns.  Secondary 
changes  :  anterior  nerve-roots.  Feripheral  nerves.  Trophic 
lésions  of  the  muscles. 

Gentlemen, — We  hâve  finished  with  tlie  form  of  progressive 
muscular  atrophy  arising  from  a  lésion  systematically  limited  to  the 
anterior  spinal  grey  substance  ;  the  moment  has  arrived  for  us  to 
discuss  some  questions  in  relation  to  spinal  amyotrophies  which 
présent  a  chronic  character,  where  the  central  lésion,  no  longer 
strictly  circumscribed,  besides  occupying  the  anterior  cornua  of 
the  cord,  occupies  also  either  the  posterior  substance  or  différent 
white  columns. 

We  hâve  agreed,  you  recollect,  to  call  the  spinal  amyotrophies  of 
this  kind  deuteropathic.  They  compose  a  complex  group,  which  as 
yet  has  been  but  little  elucidated.  But,  as  I  intimated  to  you, 
this  group  possesses  an  individuality  which  deserves  to  be  closely 
examined,  both  on  account  of  its  clinical  importance,  hitherto 
indeed  well  nigh  misunderstood,  and  likewise  because  of  the  phy- 
siological  and  anatomical  peculiarities  that  belong  to  it. 

Hère,  the  spinal  lésion  is  constituted  by  a  combination,  so  to 
speak,  of  the  obligatory  altération  of  the  anterior  grey  substance. 


AMYOTROPHIO    LATERAL    SCLEEOSIS.  181 

witli  a   symmetrical   and  primarij  sderosis   of  tJie    latéral   wJiite 
■eolumns. 


Let  us,  in  the  first  place,  consider  the  anatomo-pathological  aspect 
and,  at  the  outset,  enter  upon  a  description  of  this  singular  lésion  of 
the  white  eolumns.  Nothing,  assuredly,  can  seem  more  odd  and 
probably  unexpected  to  some  of  you  than  this  lésion,  geometrically 
circumscribed,  as  it  were,  to  a  région  of  the  white  eolumns,  which 
in  the  normal  state,  is  not  separated,  at  least  not  in  the  adult,  by  any 
perceptible  line  of  démarcation  from  the  remainder  of  the  antero- 
lateral  eolumns. 

But  surprise  soon  ceases  when  you  come  to  examine  the  facts 
supplied  by  a  study  of  the  embryo-development  of  the  spinal  cord. 
Then_,  in  fact,  you  readily  perceive  that  that  portion  of  the  antero- 
lateral  eolumns  to  which  the  inflammatory  processes  may  thus  be 
confined,  forms,  during  fœtal  life,  and  even  during  the  early  stage 
of  extra-uterine  life,  a  separate  System,  anatomically  distinct  from 
the  other  eolumns  of  the  spinal  cord. 

This,  gentleman,  is  an  almost  entirely  new  point  of  view,  at  least 
as  applied  to  pathology.  I  hâve  already  alluded  to  it,  ia  our  open- 
ing  lecture,  but  I  think  we  hâve  grounds  for  referring  to  it  now, 
in  fuUer  détail. 

The  results  which  I  am  about  to  state,  but  briefly,  are  taken  from 
the  labours  of  Budge,  Kiiffer,  L.  Clarke,  KoUiker,  JPlechsig,  and 
from  other  and,  on  some  points,  more  complète  labours  which,  at 
my  request,  were  undertaken  by  M.  Pierret,  in  the  laboratory  which 
I  direct. 

A.  The  spinal  cord  is,  you  are  aware,  only  an  incomplète  ring 
made  of  embryonic  matter,  in  the  first  stage  of  its  formation.  As 
soon  as  the  central  canal  is  closed  behind,  the  embryonic  mass 
tends  to  divide  itself  (owing  to  the  appearance  of  a  latéral  furrow 
on  either  side),  into  two  portions,  an  anterior  and  a  posterior  half 
for  each  latéral  moiety  of  the  cord.  In  this  way,  are  at  first 
sketched  out  the  rudiments  of  the  anterior  cornua  (fig.  13,  a),  and 
of  the  posterior  cornua,  of  grey  substance  (fig.  13,  b).  Towards 
the  close  of  the  first  month,  a  zone  of  white  substance  is  added, 
which  is  in  connection  with  the  nerve-roots. 


183  DEVELOPMENT   OF   SPINAL  CORD. 

Thèse  zones,  in  the  nomenclature  proposed  by  M.  Pierret,  receive 
the  following  names  : — 1°,  tlie  anterior  radicular  zones  (fig.  13,  a') 
which  go  to  constitute,  in  the  adult,  a  considérable  portion  of 
the  antero-lateral  columns  ;  and,  2°,  the  posterior  radicular  zones 
(fig.  13,  l'). 

<?-t  '        Fig.  13. — Section  of  spinal  cord  of  buman  embryo  one 

a-H  I  month  old.    a,  anterior  cornua  ;  b,  posterior  coruua  ; 

\  /  c,    central   canal  ;    d,    anterior  roots  ;    e,  posterior 

roots  ;  a',  anterior  radicular  zone  ;  b',  posterior  radi" 
e — 

I  /  cnlar  zone. 


When  the  columns  of  Goll,  not  yet  developed  at  this  period, 
shall  hâve  been  added,  they  will  ultimately  form  what  is  usually 
called  the  posterior  columns. 

The  latéral  columns  do  not  yet  exist  ;  they  are  seen  to  make  their 
appearance  towards  the  sixth  or  eighth  week  in  the  furrow,  which 
still  laterally  séparâtes  the  two  portions  of  grey  substance,  under 
the  semblance  of  two  little  masses  or  tubercles  of  embryonic  matter, 
in  which  the  nerve-tubes  do  not  show  themselves  until  very  late 
rfig.  14,0- 


PiG.  14. — Section  of  the  cord  of  human  embryo 
a  montb  and  a  lialf  old.  a,  b,  c,  &c.,  as  in  Fig. 
13.    /,  latéral  column. 


Towards  the  same  epoch,  (about  the  eighth  week,  namely),  are 
developed,  in  the  furrows  which  separate  the  posterior  radicular 
zones,  two  small  symmetrical  protubérances,  which  tend  to  unité 
together,  and  which  extend  throughout  the  whole  heigbt  of  the  cord  ; 
thèse  are  the  columns  of  Goll  (fig.  i^,m). 

At  the  same  time,  an  analogous  formation  takes  place  in  the 
furrow  which  séparâtes  the  anterior  radicular  zones,  It  relates  to 
the  small  columns  which  I  bave  proposed  to  designate  by  the  name 
of  the  columns  of  Tilrck  (Figs.  15,  16,  n'),  and  which  cannot  be 
followed,  in  the  adult,  below  the  cervical  enlargement  of  the  cord. 

Thus  is  completed  that  aggregation  of  distinct  pièces  which,  by 


DEVELOPMENT    OP    LATERAL    COLUMNS. 


183 


tlieir  union  and  more  or  less  intimate  fusion  will  form,  at  a  more 
advanced  period  of  life,  the  antero-lateral  columns,  siich  as  we  see 
them  in  the  adult. 


TiG.  15. — Sectiou  of  the  cord  of  human 
embryo  aged  two  montlis.  a,  h,  c,  as 
in  Eig.  13  ;  /,  latéral  colunin  ;  m,  de- 
velopment  of  GoU's  column  ;  n,  develop- 
ment  of  Tûrck's  columns  (the  anterior 
fascicles). 


B.  But  the  latéral  columns  being  those  which  are  alone  to  engage 
our  attention  to-day,  I  should  revert  more  particularly  to  the  cha- 


TiG.  16. — Section  of  the  cervical  eord  of 
human  embryo  aged  from  twelve  to 
fifteea  weeks.  The  letters  indicate  the 
same  things  as  above. 


racters  which  they  présent  in  the  différent  phases  of  their  évolution. 
By  the  progress  of  development  they  tend  to  fuse,  iu  front,  with 
the  anterior  radicular  zones,  and  heliind  with  the  anterior  extremity 
of  the  posterior  radicular  zones,  so  as  soon  to  become  indistinguish- 
able,  each  from  each. 

However,  even  after  birth,  in  the  new~born  child,  we  can  still, 
in  the  area  of  the  white  columns,  discern  the  région  which  belongs 
to  the  latéral  columns,  properly  so  called  by  certain  histological 
characters,  which  betray  a  comparatively  less  advanced  development. 
This  région  is  observed,  behind  an  imaginary  transversal  line  crossing 
the  commissure,  under  the  form  of  a  triangular  space  corresponding 
to  the  most  posterior  portion  of  the  antero-lateral  column.  In  this 
space  the  white  substance  is  distinguished  by  a  greyish  tint,  per- 
ceptible by  the  naked  eye.  Microscopical  examination  shows  that 
nerve  tubes,  having  medullary  cylinders  are  rare  hère  ;  and  that,  on 
the  contrary,  the  connective  matrix  is  prédominant  :  hence,  thèse 
portions  are  strongly  coloured  by  carminé,  but  scarcely  tinted  by 


T84  SECONDAEY   LATERAL    SCLEROSIS. 

osmic  acid.  Finally,  thèse  same  portions  include,  in  the  normal 
stnte,  a  certain  proportion  of  cells  studded  with  fatty  granulations, 
wliicli  represent  in  the  cord  what  M.  Parrot  désignâtes  by  tlie  name 
oï pIiT/siological  steatosis. 

I  shall  add  that,  as  shown  in  a  plate  of  Kolliker's  work,  a  more 
or  less  marked  furrow  often  dénotes,  on  the  exterior  of  the  cord  in 
young  infants,  a  séparation  between  the  latéral  columns  proper  and 
the  anterior  columns.  But,  in  the  adult,  ail  distinction  is  lost; 
liowever,  it  is  right  to  observe  that,  even  in  his  case,  the  régions 
which  correspond  to  the  latéral  columns  are  still  marked  by  the 
comparatively  small  diameter  of  the  nerve-tubes  and  by  a  certain 
prépondérance  of  neuroglia. 

Enough  has  been  said,  gentlemen,  I  hope,  tô  render  évident  the 
incontestable  independence  possessed  in  the  first  period  of  life  by 
the  latéral  columns  of  the  spinal  cord.  I  should,  however,  complète 
this  sketch  by  pointhig  out  to  you  that  this  System  is  seen  repre- 
sented  in  the  bulbus,  above  the  decussation,  by  the  anterior  pyra- 
mids,  and  also  in  the  protuberantia,  and  in  the  lower  portion  of  the 
crura  cerebri.  Now,  thèse  régions  of  the  isthmus  of  the  encephalon 
and  bulbus  which  are  in  relation  with  the  latéral  columns,  are  dis- 
tinguished,  like  the  latter,  in  the  fœtus,  by  late  development,  which 
is  but  imperfectly  accomplished  at  the  time  of  birth. 

II. 

The  individuality,  the  autonomy  of  the  latéral  columns,  which  has 
been  already  rendered  évident  by  the  foregoing  remarks,  is  again 
distinctly  exhibited  when  we  examine  the  phenomena  which  pertain 
to  the  sphère  of  pathology.  It  is  not  unknown  to  you — for  the 
subject  occupied  us  last  year  —  that,  after  a  unilatéral  lésion  of 
certain  departments  of  the  encephalon,  an  entire  half  of  the  System 
of  latéral  fibres,  alike  in  the  crus,  in  the  protuberantia,  in  the 
bulbus,  and  throughout  the  whole  height  of  the  spinal  cord,  under- 
goes,  separately  and  singly,  a  consécutive  lésion,  which  soon  shows 
itself  histologically  by  characters  peculiar  to  sclerosis  of  the  nerve 
centres.  In  the  isthmus,  and  in  the  bulbus,  the  fasciculated  sclerosis 
may  be  followed  down  to  decussation,  on  the  same  side  as  the  céré- 
bral lésion.  Beneath  the  point  of  decussation  it  occupies,  on  the 
contrary,  the  other  side  of  the  cord. 

This  lésion  of  the  System  of  latéral  fascicles  is,  in  such  a  case,  ab- 
solutely  isolated  ;  it  is,  in  particular,  not  accompanied,  at  least  as  a 


PKOTOPATHIC  LATERAL  SOLEROSIS.         185 

gênerai  rule,  by  any  altération  of  the  anterior  grey  substance  or  of 
tlie  spinal  motor  roots.  In  connection  witli  tliis  subject  I  would  re- 
mind  you  that  the  hemiplegia  with  contracture,  wliich  coexists  with 
tliis  lésion,  is  remarkable  for  integrity  of  nutrition  in  the  paralysed 
muscles,  so  long  as  functional  inertiahas  not  beenovermuch  prolongea. 

In  a  case  where  the  primary  cérébral  lésion  should  simultaneously 
occupy  corresponding  points  in  botli  hémisphères,  then  the  System  of 
latéral  columns  would  naturally  be  affected  on  both  sides,  right  and 
left,  throughout  its  whole  extent,  in  the  isthmus  as  well  as  in  the 
bulbus  and  spinal  cord.  In  tliis  hypothesis,  which  has  been 
more  than  once  realised,  we  should  consequently  hâve  to  deal  with 
a  symmetrical  latéral  sclerosis,  consécutive  on  cereiral  lésion. 

But  a  total  symmetrical  sclerosis  of  the  latéral  columns  may 
supervene  protopathically,  primarily,  without  dependence  on  any 
encephalic  lésion  whatever.  This  is  a  fact  which  L.  Tiirck,  in  1856, 
and  which  I  myself,  ten  years  later,  hâve  rendered  évident,  and 
which  it  is  proper,  at  présent,  to  set  out  in  a  prominent  manner. 

Hère,  two  cases  may  présent  themselves  : 

1°.  Primary  symmetrical  sclerosis  alone  is  seen  to  exist,  without 
being  complicated  with  any  lésion  of  the  anterior  grey  substance  ; 
the  most  salient  feature  in  the  syndromus  in  connection  with  the 
lésion,  thus  established,  is  a  paresis  of  the  limbs,  especially  of  the 
lower  extremities,  marked  by  more  or  less  intense  contracture  of  the 
muscles,  which  long  préserve  ail  the  characters  indicative  of  normal 
nutrition.  This  lésion  of  the  spinal  cord  is  rather  frequently  seen, 
amongst  other  matters,in  the  course  of  progressive  gênerai  paralysis — 
a  coïncidence  which  has  been  particularly  noticed  by  H.  Westphal. 

2°.  But  it  happens  rather  frequently  that  an  altération  of  the 
grey  substance  is  associated  with  the  symmetrical  lésion  of  the  latéral 
columns.  Now,  the  combination  of  thèse  two  orders  of  altération 
exactly  constitutes  the  anatomical  substratum  of  the  pathological 
form  to  which  I  désire  to  call  your  attention.  The  symptoms  of 
progressive  amyotrophy  are  then  associated  with  those  arising  from 
latéral  sclerosis. 

III. 

We  hâve  now  to  study  more  closely,  from  an  anatomical  point  of 
view,  the  altérations  in  question.  In  the  following  description  we 
shall  hâve  to  examine  successively — 1°,  the  lésions  presented  by  the 
■System  of  latéral  fascicles  in  the  several  régions  of  the  cord,  the 


186 


PATHOLOGICAL    ANATOMY. 


buîbus,  and  in  the  isthmus  of  the  encephalon  ;  %,  the  concomitant 
lésions  of  the  grei/  substance  in  the  same  departments  of  the  nerve 
centres  ;  3°,  the  consécutive  lésions  of  the  anterior  roots  and  of  the 
sjnnal  nerves  ;  4°,  lastly,  the  iroplùc  lésions  of  the  muscles. 

With  respect  to  the  first  point  I  shall  be  brief,  because  I  assume 
your  acquaintance  with  consécutive  scléroses  of  cérébral  origin,  the 
anatomical  characters  of  which  are  verj  nearly  the  same  as  those  of 
primary  scléroses.  I  will  confine  my  remarks  to  noting  the  following 
points  : 

A.  Let  us  consider,  in  the  first  ])lace^  what  passes  in  the  corcl. 
a.  When  transverse  sections  of  the  cervical  enlargement  are 
examined,  it  is  found  that  the  symmetrical  altération  hère  com- 
prises a  greater  extent,  in  breadth,  than  anywhere  else.  Thus,  the 
région  invaded  by  sclerosis  extends  forward  to  a  level  with  the 
outer  angle  of  the  anterior  cornu,  and  even  beyond  it.  Behind, 
it  is  nearly  bounded  by  the  posterior  grey  substance.  On  the 
outer  side,  however,  it  is  constantly  separated  from  the  cortical  layer 
of  the  cord  by  a  tractus  of  uninjured  white  substance  (fig.  17,  a,  a). 
See  also  PL  IV,  figs.  i,  2,  3  ;  PI.  V,  figs.  i  and  1). 


Fig.  17. 


Fig.  18. 


Fig.  19. 


À     A 


Fig.  17.— Trans verse  section  of  the  spinal  cord,  taken  from  the  middle  portion 
of  the  cervical  enlargement. 

Fig,  18. — Transverse  section,  taken  from  the  middle  of  the  dorsal  région. 

FiG.  19. — Transverse  section,  taken  from  the  middle  of  the  lurabar  enlarge- 
ment. 

Ail  other  parts  of  the  white  columus  are  left  unharmed,  with  the 
exception  of  the  small  fascicles  of  Tûrck  which,  in  certain  cases, 
are  symmetrically  aiîected.  Thèse  fascicles,  I  may  say  in  passing, 
seem  to  belong  to  the  same  System  as  the  latéral  fascicles. 

b.  In  the  dorsal  région,  the  lésion  is  more  circumscribed  (fig.  18). 
In  front,  it  does  not  even  reach  an  imaginary  line  crossing  the  com- 
missure. Externally  it  approaches  the  cortical  zone,  from  which  it 
is  only  separated  by  a  very  thin  strip  of  uninjured  white  substance. 

c.  Pinally,  in  the  lumbar  région  the  lésion  is  still  less  extensive. 


LESIONS    OP    MOTOE    NEUVE-CELLS.  187^ 

It  barely  occupies  the  posterior  fourth  of  tlie  latéral  columns.  It 
is  worth  noticing  thai,  externally,  it  reaches  to  the  cortical  zone 
(fig.  19). 


A'    ^  A 

Fig.  20. — Transverse  section  of  the  bulbus,    from   the   mid-portion  of  the 
olivary  bodies.     a.  a.  Anterior  pyramids,  affected  by  sclerosis. 

B.  Secondlvj  wliat  do  we  find  in  the  hulhus  ?  Tlie  lésion  shows 
itself  hère  by  an  invasion  of  the  anterior  pyramids^  throughout 
their  whole  height  (fig.  20).  Above,  in  the  lower  part  of  the  pro- 
tuberantia,  the  lésion  may  be  followed  upward  so  long  as  the  fibres 
coming  from  the  pyramids  are  still  united  in  bundles  ;  but,  higher 
again,  when  thèse  fibres  scatter,  they  are  readily  lost  sight  of. 

Some  authors  hâve  traced  the  lésions  of  primary  latéral  sclerosis 
even  into  the  foot,  or  lower  portion,  of  the  crus  cerebri  ;  but  it  is 
not  known  how  it  ends,  that  is,  so  far  as  the  brain  is  concerned. 
However,  the  internai  capsule  which,  in  part,  seems  only  to  be  a 
prolongation  of  the  lower  portion  of  the  crus,  is,  notwithstanding, 
not  invaded. 

IV. 

This  terminâtes  what  we  hâve  to  say  in  référence  to  altérations 
of  the  white  columns.  It  now  becomes  our  duty,  gentlemen,  ta 
notice  those  which  belong  to  the  grey  substance. 

They  differ  in  nothing  essential  from  those  which  we  studied  in 
connection  with  proiopaili'ic  spmal  muscular  atropliy.  This  means 
that  hère,  also,  they  are  systematically  localised  in  the  anterior  grey 
cornua.  In  that  région,  just  as  in  the  former  case,  they  afi^ect  both 
the  neuroglia  and  the  motor  nerve-cells  which  are,  more  or  less 
numerously,  degenerated,  atrophied,  or  even  completely  destroyed 
(see  PI.  IV,  fig.  4). 

It  is  the  rule  that  the  altération  of  the  grey  substance  sliall  not 
exceed  the  area  of  the  anterior  cornua;  this  peculiarity  is  rendered 
especially  évident  by  the  perfect  integrity,  as  has  been  frequentlj 


188  LESIONS    OF   BULBUS. 

verified,  of  the  cell-groups  of  Clarke^s  column  in  the  dorsal 
région. 

a.  The  altération  of  the  spinal  grey  substance,  in  ail  the  cases 
wliich  I  bave  observed,  preponderates  in  the  cervical  région  of  the 
spinal  cord  ;  it  is  also  often  very  marked  in  the  dorsal  région  ;  but 
it  tends  to  become  attenuated  as  we  descend  towards  the  lumbar  en- 
largement.  This  disposition  of  the  lésion  is  correlated  to  a  circum- 
stance  which  I  shall  not  fail  to  bring  out,  prominently,  in  the  clinical 
description  ;  it  is  this,  namely,  that  muscular  atrophy,  in  the  noso- 
graphical  form  which  occupies  us,  rarely  affects  the  inferior  extre- 
mities.  Thèse  limbs  are  paralysed  and  stricken  with  contracture  at 
a  very  early  period,  which  the  existence  of  the  latéral  sclerosis 
accounts  for,  but  their  muscles  are  either  not  atrophied  or  but  little 
wasted,  in  comparison. 

h.  The  altérations  of  the  grey  substance  of  the  spinal  cord,  as 
"well  as  those  of  the  white  columns,  hâve  their  pendant  in  the 
.  medulla  oblongata.  You  are  not  unaware,  gentlemen,  that  there 
exists,  in  this  part  of  the  nerve  centres,  a  certain  number  of  nuclei 
of  grey  substance,  which  are  regarded  as  analogous  to  the  anterior 
cornua  of  the  cord,  and,  consequently,  as  being  the  région  whence 
the  motor  bulbar  uerves  originate.  This  supposition  is  scarcely 
open  to  doubt,  ])articularly  with  respect  to  the  nuclei,  whence 
originate  the  hyjioglossus,  the  spinal,  and  even  the  facial  nerves. 
ISFow,  to  speak  only  of  the  first  named,  the  great  multipolar  cells 
which  compose  it  and  which,  morphologically,  are  so  closely  ana- 
logous to  the  great  motor  cells  of  the  cord,  thèse  cells,  I  repeat, 
become  atrophied  or  destroyed  simultaneously  with  the  sclerosis  of 
the  neuroglia  which  encloses  them  (fig.  31).  But,  for  the  présent 
moment,  I  shall  content  myself  with  merely  indicating  the  fact,  in 
order  not  to  leave  wholly  in  the  shade  an  entire  corner  of  the 
picture,  and  an  interesting  one  besides.  I  will  return  to  the  subject 
when  studying  bulbar  amyotrophies  and  labio-glosso-laryngeal 
>paralysis. 

V. 

Ail  that  now  remains  for  me  is  to  make  you  acquainted  with  the 
changes  which  are  produced,  consecutively  on  the  foregoing,  in  the 
anterior  roots  and  in  i\\e,  iperlpheral  nerves.  I  hâve  merely  to  repeat 
hère  what  I  hâve  already  said  with  respect  to  protopatliic  spinal 
muscular  atrophy.  Nerve-tubes  entirely  devoid  of  medullary  matter 


TROPHIC  LESIONS    OP  MUSCLES. 


189 


are  rare  in  the  roots  as  well  as  in  tlie  peripheral  nerves.     Granular 
tubes  are  in  minority.     Most  of  the  nerve-tubes  are  preserved  ;. 


FiG.  21. — Trausverse  section  of  tlie  bulbus,  on  a  level  with  the  middle  of  the 
nucleus  of  the  hypoglossus.  a,  b  (to  the  right  of  the  imagiuary  line  r,  k,'), 
represent  the  normal  condition  ;  a,  nucleus  of  the  hypoglossus,  composed 
of  a  cluster  of  some  thirty  great  multipolar  cells  ;  v,  a  vessel  which  bounds 
the  nucleus  in  front  and  on  the  inner  side  ;  c,  floor  of  the  fourth  ventricle  • 
D.  fasciculus  teres  ;  B,  nucleus  of  pneumogastric  nerve.  a',  b',  &c.  (to 
left  of  the  imaginary  line  r,  k')  represent  the  same  parts  in  a  case  of 
amyotrophic  latéral  sclerosis.  It  is  seen  that  hardly  five  or  six  nerve-cells 
are  left  uninjured  in  the  area  of  the  nucleus  of  the  hypoglossus.  k',  fasci- 
culus teres.  b',  Nucleus  of  the  pneumogastric,  exhibiting  no  perceptible 
change. 

nearly  ail  of  them,  however^  hâve  undergone  a  certain  degree  of 
simple  atrophy.  This  is  a  fact  which  shall  be  brought  out,  pro- 
minently,  when  we  treat  of  the  pathogeny  of  consécutive  muscular 
lésions. 


VI. 

I  may  also  deal  very  briefly  with  the  tropkic  lésions  qf\the 
muscles.  They  do  not  differ,  in  any  essential  manner,  from  those 
which  are  met  with  in  primary,  spinal  amyotrophy.  Still,  the 
inflammatory  character  of  the  lésion  has  appeared  lo  me  more 
marked  in  amyotrophic  latéral  sclerosis.  Thus,  the  hyperplasia  of 
the  perimysium  is  more  manifest  and,  in  one  case,  I  even  found^ 


190 


TEOPHIC    LESIONS  OF   MUSCLES. 


with  M.  Debove,  tlie  interstitial  connective  tissue  infiltrated  witli  a 
considérable  number  of  leucocytes  in  some  places. 

I  would  note  particularly  that  interstitial  lipomatosis  of  the 
muscles  is  produced,  in  the  amyotrophy  connected  with  latéral 
sclerosis,  just  as  in  pure  amyotrophy.  This  fact  is  interesting  in 
connection  with  the  tongue,  the  muscles  of  which,  in  amyotrophic 
sclerosis,  waste  equally  with  tliose  of  the  extremities^  in  consé- 
quence of  the  altération  of  the  cells  of  the  hypoglossal  nucleus. 
Nevertheless,  the  tongue,  in  such  cases,  may  almost  entirely  pré- 
serve its  normal  volume^  and  not  présent,  on  its  surface,  the  con- 
volutions  and  wrinkles,  animated  by  vermicular  movements,  which 
are  often  observed  there.  In  thèse  diverse  circumstances,  its 
muscular  fibres  are  atrophied.  This  préservation  of  the  form 
and  volume  of  the  organ  is  explained  in  the  cases  I  refer  to,  by 
interstitial  lipomatosis.  In  one  of  thèse  cases  I  hâve  remarked, 
with  M.  Debove,  the  existence  of  a  kind  of  hypertrophie  cirrhosis, 
produced  by  the  excessive  végétation  of  the  perimysium,  both 
internallv  and  externally  (figs.  32,  23). 


FiG.  22. — Section  of  longue  ;  normal  condition. 

After  this  description  of  the  lésions  peculiar  to  latéral  amyotro- 
phic sclerosis,  it  is  our  intention  to  make  you  acquainted  with  the 
principal  symptoms  which  betray  its  existence  during  life,  with  a 


TEOPHIO    LESIONS    OF    MUSCLES. 


191 


view  to  détermine,  as  closely  as  possible,  the  physiological  connec- 
tion whicli  attaches  the  lésions  to  tlie  symptoms. 


EiG.  23. — Section  of  tongue  in  a  case  of  amyotrophic  latéral  paralysis,  with 
labio-glosso-laryngeal  paralysis. 

It  may  be  stated,  in  a  very  gênerai  manner,  tbat  the  symptoms 
to  wliicli  I  allude  are  of  two  orders  :  one  set  is  in  corrélation  with 
the  symmetrical  altération  of  the  latéral  columns,  the  other  dépends 
upon  the  concomitant  lésion  of  the  grey  substance.  This  is  what  I 
shall  endeavour  to  demonstrate  at  our  next  meeting. 


LECTUEE   XIII. 
ON  AMYOTROPHIC  LATERAL  SCLEROSIS.    SYMPTOMATOLOGY. 

SuMMARY. — Facts  which  form  tlie  basis  of  tke  symptomatology  of 
aviyolroplàc  latéral  sclerosis.  Personal  oiservations.  Cor- 
roborative  cases. 

Différences  which  clinically  separate  amyotrophic  latéral 
sclerosis  front  protopathîc  spinal  mîiscular  atrophy. 

Symptoms  common  to  hoth  affections.  Progressive  amyo- 
trophy,  fibrillary  contractions,  préservation  of  electric  con- 
tractility. 

Symptoms  peculiar  to  amyotropîdc  latéral  sclerosis.  Pré- 
dominance qf  motor  paralysis.  Permanent  spasmodic  contrac- 
ture. Absence  of  sensory  disorders.  Paralytic  déformations  ; 
attitude  qf  hand.  Tremulation  of  upper  extremities  in  pur- 
posed  movements.  Modes  of  invasion.  Cervical  paraplegia. 
Livasion  of  inferior  extremities.  Characteristics  of  the  co7i~ 
tracture.  Bulbar  phenomena  ;  difficulty  of  déglutition.  Im~ 
peded  speech.  Paralysis  of  the  vélum  palati,  ofthe  orbicularis 
oris,  8fc.     Grave  disorders  qf  respiration. 

Summary  qf  symptoms,  Prognosis.  Pat/iological  phy- 
siology. 

I. 

Gentlemen, — After  describing  the  necroscopic  changes  peculiar 
to  latéral  amyotrophic  sclerosis  it  becomes  us  now  to  enliven  the 
picture  by  showing  you  the  séries  of  symptoms  which,  during  life, 
are  correlated  to  thèse  lésions. 

I  trust,  gentlemen,  to  establish  that  tins  symptomatic  group  is 
striking  and  characteristic  enough  to  be  readily  distinguished  from 
that  which  dépends  on  the  circumscribed  altération  in  the  anterior 
spinal  grey  substance.  It  will  be,  also,  easy  for  me,  I  believe,  after- 
wards  to  draw  a  distinct  line  of  démarcation  between  latéral  amyotrophic 


PERSONAL    OBSERVATIONS.  193 

»clerosis  and  the  otlier   forms  of  deuteropathic   spinal  miiscidcn- 
atrophy. 

1°.  At  the  outset,  I  ouglit  to  mention  that  the  observations 
which  are  to  serve  as  the  basis  of  my  description  are  as  yet  not 
numerous,  being  only  a  score  at  most.  But,  it  may  be  added  that 
the  same  thing  occnrred,  on  a  former  occasion,  in  connection  with 
progressive  locomotor  ataxia.  And  yet  the  clinical  picture  traced 
by  Duchenne  (de  Boulogne)  with  the  help  of  a  few  facts,  some 
twenty  years  ago,  has  not  lost  by  âge.  It  still  remains,  at  the 
présent  moment,  such  as  it  was  in  ail  essential  particulars,  without 
having  undergone  any  serions  modifications.  May  the  description 
which  I  am  about  to  offer  you  of  amyotropUc  latéral  sclerosis 
expérience  the  same  destiny  ! 

Most  of  the  facts,  whose  assistance  I  can  rely  upon,  hâve  been 
collected  by  myself  or  by  my  students,  in  the  Salpêtrière  Hospital. 
At  the  commencement^  the  observations  were  chiefly  noted  from  the 
standing  point  of  pathological  anatomy.i  Nevertheless  the  symptoms 
had  almost  always  been  set  down  with  some  care.  Hence,  at  a  given 
period,  it  became  possible,  on  comparing  the  dijBFerent  observations, 
to  perceive  a  certain  number  of  fundamental  features,  which  after- 
wards  enabled  us  to  recognise  the  disease  during  life.  Such,  also,  has 
been  the  history  of  disseminatecl  sclerosis  ;  for  a  long  time,  only  the 
singular  lésions  which  characterise  it  anatomically  were  known  to  us. 
To-day,  it  takes  rank,  in  gênerai  clinical  practice. 

In  addition  to  my  own  facts,  I  hâve  found  in  différent  publica- 
tions some  more  or  less  complète  observations,  which  belong  in  every 
particular,  to  the  pathological  form  in  question,  and  I  hâve  conse- 
quently  turned  them  to  advantage. 

I  will  mention,  firstly,  amongst  facts  of  this  group.  Cases 
II  and  IV  of  the  excellent  memoir,  published  in  1867  by  M. 
Duménil  (of  Eouen),  on  progressive  muscular  atrophy,  in  the 
^  Gazette  hebdomadaire.'  Then,  I  would  refer  to  three  observations 
reported  by  Leyden.  They  bave  published,  as  examples  of  holhar 
paralysis,  in  the  '  Archiv  fur  Psychiatrie,'  edited  by  H.  Westphall.- 

ï  The  observations,  foUowed  by  autopsy,  collected  by  me  in  the  Salpêtrière 
Asylum  are  ûve  in  number.  I  shall  give  a  summary  of  them  further  on. 
Two  of  thèse  observations  hâve  been  published,  with  détails  :  one  by  M. 
Joffroy  and  myself  (' Arch,  de  Physiologie,'  1869,  p.  356);  the  other,  in  the 
same  journal  (1871-72,  p.  509),  by  M.  Gombault. 

'  E.  Leyden,  "  Ueber  progressive  Bulbàr-paralysie,"  in  '  Archiv  fur  Psychia- 
trie,' ii  Ed.,  p.  648  ;  Obs.  I,  uud  p.  657  ;  Obs.  II,  iii  Ed.,  p.  338. 

VOL.   II.  13 


194  COKEOBOEAÏIVE   CASES. 

I  liave  also  to  mention  a  case  inserted  by  H.  Otto  Barth  in 
Wunderlich's  journal,  under  tlie  title  of  AtropJiia  museulorunt 
lîpomatosa}  The  author,  little  careful  of  nosographical  rules, 
seems  to  think  that  he  had  before  him  an  example  of  pseudo- 
hypertrophic  paralysis,  as  understood  by  M.  Duchenne  (de  Bou- 
logne). In  reality,  the  autopsy,  which  was  raade  witli  much 
care,  aflFords  superabundant  évidence  tbat  the  disease  in  question 
was  primary  symmetrical  sclerosis  of  the  latéral  columns  with  con- 
comitant lésions  of  the  anterior  greij  substance.  A  case,  recorded 
by  Dr.  Hun,^  another  published  by  Mr.  S.  Wilks,  in  '  Guy's 
Hospital  Eeports,'  ^  are  also,  in  my  judgment,  examples  of  amy- 
otrophic  latéral  sclerosis.  Finally,  I  wouid  also  include,  in 
the  same  category,  two  observations  recently  published  by  Drs» 
Lockhart  Clarke,*  and  R.  Maier,  of  Friburg.^ 

In  concluding  this  review  of  corroborative  articles,  I  should  men- 
tion, gentlemen,  that  M.  Duchenne,  de  Boulogne,  in  the  new  édition 
of  his  book,6  bas  opened,  under  the  heading  of  Suhacnte  diffuse 
gênerai  spinal  paralysis,  a  chapter  in  which  appears  one  of 
the  cases,  noted  in  my  wards  at  the  Salpêtrière  in  connec- 
tion with  amyotrophic  latéral  sclerosis.  This  chapter  also  con- 
tains  a  large  number  of  heterogeneous  éléments  which  could  not 
be  classed  elsewhere.  The  greater  number  of  the  deuteropathic 
chronic  spinal  amyotrophies  are  grouped  together  under  the 
same  dénomination.  Evidently,  this  could  only  be  a  tentative 
chapter,  a  sort  of  caput  mortuum,  which  requires  to  be  entirely 
remodelled. 

To  those  amongst  you  who  may  be  désirons  of  examining  de  visic 
the  symptoms  of  amyotrophic  latéral  sclerosis,  I  would  mention 
that  there  exists  at  the  présent  moment,  in  La  Charité,  under  the 
care  of  Dr.  Woillez,  a  poor  mason,  aged  44,  who  présents,  at  least 

^  0,  Barth,  "  Zur  Kenntniss  der  Atrophia  musculorum  lipomatosa,"  in 
'  Archiv  der  Heilkunde,'  1871,  p.  121. 

-  'American  Journal  of  lusanity,'  Oct.  2ud,  1871,  and  ' Centralblatt,'  1872, 
p.  429. 

^  Vol.  XV,  I.  46,  and  '  Centralblatt,'  p.  239,  ISlo.  15,  1870. 

■•  J.  Lockhart  Clarke,  '  Progressive  Muscular  Atrophy,  accompanied  by 
Muscular  Rigidity  and  Contraction  of  Joints;  examination  of  the  Braiu  and 
Spinal  Cord,"  in  '  Medico- Chirurgie.  Transactions,'  t.  ivi,  p.  103. 

'  E..  Maier,  ''  Ein  Fall  von  fortschreitender  Bulbàr-paralysie,"  in  '  Yircliow's 
Archiv,'  6ie  Ed.,  ler  Hei't,  p.  i. 

*>  '  Electrisation  localisée,'  3e  édition,  1872,  p.  469. 


PROTOPATHIO    AND   DEUTEROPATHIC   AMYOTROPHIES.    195 

in  my  opinion,  ail  tlie  f  and  ameutai    clinical  characters  of   this 
affection.' 

II. 

1°.  One  of  the  first  distinctive  features  which,  of  itself,  radically 
séparâtes  amyotrophic  latéral  sclerosis  from  primary  muscular 
atrophy  is  the  comparative  rapidity  of  its  évolution,  considérée! 
from  the  invasion  of  the  first  symptom  to  the  fatal  end.  This  does 
not  usually,  ou  an  average,  delay  more  than  three  years  and  it  may 
supervene  much  sooner,  at  the  end  of  one  year,  for  instance  ;  whilst 
patients,  affected  by  protopathic  spinal  progressive  muscular  atrophy 
may  survive,  as  y  ou  are  aware,  for  eight  or  ten,  and  even  for  fifteen 
and  twenty  years. 

2°.  During  this  comparatively  short  period,  it  is  the  rule  that 
the  four  extremities  shall  be  successively,  and  within  a  brief  space,, 
ail  stricken  with  paralysis  accompanied  by  atrophy,  or  with  paralysas 
only  in  the  lower  limbs.  The  patient  after  some  months,  a  y  car  or 
two,  three  years  at  most,  is  confined  to  his  bed,  and  more  or  less 
completely  deprived  of  the  use  of  his  limbs.  But,  in  addition,  to  judge 
at  least  from  ail  the  cases  which  I  hâve  collected,  we  regularly  find  the 
disease  extending  to  the  bulbus,  and,  it  is  nearly  always  to  the 
paralysis  of  the  bulbar  nerves,  more  especially,  of  the  hypoglossus 
and  pneumogastric  that  the  phenomena  which  détermine  death  are 
to  be  attributed.  This  forms  a  contrast  with  what  we  know  con- 
ceming  common  progressive  muscular  atrophy,  since  hère,  according 
to  the  statistics  furnished  by  Dr.  Duchenne,  atrophy  of  the  muscles 
animated  by  the  bulbar  nerves  has  been  found  to  appear  only 
thirteen  times  in  159  cases. 

3°.  The  data  obtained  from  an  examination  of  etiologieal  influences 
are  not  of  remarkable  importance,  so  far  as  we  hâve  yet  gone, — 
this,  however,  is  easily  understood  on  account  of  the  small  number  of 
particulars  which  can  be  tabulated.  I  shall  confine  myself  to  the 
following  observations  : 

1  The  patient  Las  succumbed,  since  this  lecture  was  delivered,  after  the 
manifestation  of  bulbar  symptoms.  The  autopsy  was  made  by  M.  Voisin, 
acting  clinical  clerk.  An  examination  of  the  cord  made  by  M.  Gombault, 
"préparateur"  for  the  course  of  pathological  anatomy,  lias  demonstrated  tbe 
existence  of  symmetrical  latéral  scferosis,  with  atrophy  of  the  motor  nerve- 
cells  in  the  cervical  région  of  the  cord  and  in  the  nuclei  whence  originate  the 
bulbar  nerves.  The  prepared  sections,  in  connection  with  this  case,  hâve  heen 
shown  at  the  Cours  Pratique  de  la  Faculté. 


Î96  DIFFERENTIAL    CHARACTEES. 

Hereditary  influence  lias  not  been  mentioned  in  our  cases.  As 
regards  âge,  tlie  disease  makes  its  appearance  at  âges  varying 
from  26  to  50  years.  Females  are  more  frequently  affected 
tlian  moles,  whicli  is  contrary  to  what  is  remarked  in  cases  of  pro- 
topathic  atrophy  ;  but  it  is  necessary  to  notice  tbat  most  of  the 
cases  of  amyotropbic  latéral  sclerosis  bave  been  noted  in  the  Sal- 
pêtrière,  that  is  to  say,  in  an  asylum  to  which  only  women  are 
admitted. 

Perbaps  a  tbird  of  tbe  patients  attribute  tlie  development  of  the 
disease  to  the  influence  of  colcl  and  clamp,  to  which  their  business 
exposed  them.  The  mason,  in  La  Charité^  rightly  or  wrongly,  lays 
the  blâme  on  a  fall  which  he  got,  two  or  three  months  before  the 
appearance  of  the  first  symptoms,  the  immédiate  resuit  of  which 
was  a  fracture  of  the  clavicle. 

I  will  not  delay  longer  over  the  etiological  aspect  of  the  question, 
which  can  only  be  profitably  considered  in  a  more  or  less  distant 
future.  Etiology  is  based  especially  upon  a  wide  foundation  of 
figures,  and  we  are  yet  far  from  being  able  to  furnish  them. 

4°.  It  is  now  time,  gentlemen,  to  come  to  the  analysis  of  the 
symptoms.     Thèse  symptoms  are  of  two  orders  : 

A.  Some  are  common  both  to  progressive  amyotrophy  and  to 
amyotrophy  from  latéral  sclerosis  ;  thèse  are  : — a, progressive  atrophy, 
invading  the  muscular  masses  ;  h,  fibrillary  contractions  which  are 
especially  seen  in  the  active  period  of  the  atrophy  ;  c,  the  préser- 
vation of  faradaic  contractility  which  the  wasting  muscles  exhibit 
to  the  last  moment. 

B.  Other  symptoms  are  quite  foreign  to  protopathic  spinal  amyo- 
trophy ;  first  comes  motor  impotence,  promptly  developed,  which, 
if  it  do  not  always  précède  the  atrophy,  is  often  strikingly  évident 
even  when  the  latter  is  not  yet  well  marked.  Speaking  generally, 
we  may  say  that,  in  protopathic  amyotrophy ,  motor  impotence 
dépends  in  a  great  measure  on  the  atrophy  of  the  muscular  masses, 
whilst  in  latéral  sclerosis,  paralysis  certainly  dominâtes  the  scène  ;  the 
atrophy  of  the  muscles  is  then  often  but  a  consécutive  or  even 
only  an  accessory  phenomenon. 

Hère  again  is  a  new  feature  of  distinction.  The  extremities, 
more  or  less  deprived  of  their  natural  movements,  are  usually  in 
latéral  sclerosis  affected  by  habituai  rigidity,  resulting  from  what  is 
called  permanent  spasmodïc  contracture.  This  phenomenon  is 
absolutely  foreign  to  primary  atrophy. 


INVASION   OF   AMYOTROPHIC   LATERAL    SCLEROSIS.       197 

Lastly,  in  the  latter  disease,  tlie  absence  of  any  sensory  disorders 
is  the  rule,  whilst  in  the  former  it  is  common  enough  for  the  patients 
to  expérience,  or  to  hâve  experienced,  in  the  afFected  members  : — i", 
more  or  less  acute  spontaneous  pains,  numbness,  or  formication  in 
the  afFected  limbs;  and  also  pcdns  provoJced  hy  pressure  or  traction 
qf  the  muscîilar  rtiasses.  I  lay  emphasis  on  the  latter  phenomenon, 
which  I  hâve  not,  as  yet,  observed  in  protopathic  progressive 
amyotrophy. 

III. 

But  the  real  characteristics  of  the  pathological  form,  whose 
description  engages  our  attention,  are  especially  rendered  évident 
when  we  consider  the  mode  of  distribution,  of  concaténation,  and  of 
évolution  of  the  symptoms. 

a.  The  disease  begins,  in  the  great  majority  of  cases,  by  the 
upper  extremities,  without  fever,  most  frequently  without  any 
perceptible  indisposition,  sometimes  after  sensations  of  formication 
and  numbness. 

From  the  outset,  the  complaint  is  one  of  diminished  motor  power, 
and  when  this  first  seriously  attracts  the  attention  of  the  patient, 
the  muscles  of  the  affected  members  generally  présent,  even  at  this 
period,  a  certain  degree  of  emaciation.  But  neither  the  latter,  nor 
the  paresis,  is  usually  limited  to  a  circumscribed  région  of  the  limb, 
to  some  muscles  of  the  hand  or  forearm,  for  instance  ;  it  extends  a 
little  everywhere,  in  a  uniform  raanner,  so  to  speak,  from  the  ex- 
tremity  of  the  member  to  its  root.  We  no  longer  observe,  hère, 
that  individual  atrophy  of  the  muscles,  which  we  noticed  in  connec- 
tion with  common  muscular  atrophy;  on  the  contrary,  we  see  a 
kind  of  gênerai  emaciation  of  atrophy,  en  r/iasse. 

It  never  attains,  in  the  commencement,  a  degree  of  intensity 
sufhcient  to  account,  of  itself,  for  the  motor  impotence.  In  short, 
we  hâve,  in  this  case,  to  deal  with  a  true  paralysis  accompanied,  or 
rather  followed,  by  atrophy,  more  or  less  rapid  and  more  or  less 
generalised,  of  the  entire  extremity. 

Besides,  the  atrophied  or  wasting  muscles  are  stirred  by  fibrillary 
movements,  which  are  often  very  marked,  and,  as  in  simple  atrophy, 
they  préserve  the  faradaic  contractility  almost  intact,  so  long  as 
the  atrophy  has  not  attained  its  extrême  limit. 

b.  In  addition  to  the  emaciation  of  the  muscles,  the  paralysed 
and  wasted  members  soon  become  the  seat  of  more  or  less  marked 
déformations  or  déviations. 


198 


PARALTTIC   DEFORMATIONS. 


The  déformations  dépend  undoubtedly,  in  part,  on  tlie  prédomi- 
nant action  of  certain  muscles  whicli  are  less  intenselj  affected  than 
ihe  rest  {^paralytic  déformations).  But  tins  is  iiot  tlie  case  as 
regards  the  majority  of  them  ;  the  déviations,  as  a  rule,  are  due  to 
the  spasmodic  contraction  of  certain  muscles,  to  a  true  contracture 
which  renders  a  large  number  of  articulations  rigid.  Thus,  to  speak 
'first  of  the  upper  extremity  only,  hère  is  the  attitude  which  it 
usually  présents  (fig.  24). 


ÏIG.  24. — Attitude  of  the  forearm  and  hand  in  the  case  of  the  patient  Tr — , 
aged  fifty-eight,  suffering  from  amyotrophic  latéral  sclerosis. 

The  arm  is  extended  close  along  the  body  and  the  shoulder- 
muscles  resist  any  effort  made  to  remove  it  from  that  position. 

The  forearm  is  semi-flexed,  and  also  pronated  ;  it  is  impossible  to 
supinate  and  extend  it  without  using  force  and  provoking  pain. 

The  same  thing  is  found  at  the  wrist  which  is,  also,  frequently 
in  semi-flexion,  whilst  the  fingers  are  bent  in  upon  the  palm  of 
the  hand  (fig.  34). 

Thèse  constrained  attitudes,  and  the  pain  caused  by  any  effort  to 
alter  them,  taken  in  connection  with  the  almost  gênerai  and  uni- 
form  emaciation  of  the  muscles  supervening  within  a  few  months, 
would  in  some  sort  suffice  to  show  that,  in  such  cases,  we  hâve  uot 
to  do  with  primary  spinal  muscular  atrophy. 

I  should  not  forget  to  mention  another  peculiarity.  Sometimes, 
in  latéral  sclerosis,  the  paretic  upper  extremities,  contractuxed  and 
âtrophied,  hâve,  nevertheless,  still  preserved  some  power  of  move- 


PERiTANENT  CONTRACTURE.       CERVICAL  PARArLEGIA.     199 

ment.  Well,  in  making  this  movement,  for  instance,  in  raising  the 
entire  arm,  we  see  the  member  taken  with  a  tremidatlon,  which 
recalls  that  observed  in  disseminated  sclerosis,  and  also  in  certain 
patients  who,  consecutively  on  a  blood-clot  cérébral  lésion,  are 
stricken  with  hemiplegia  and  contracture.  This  tremulation,  in  the 
two  latter  cases,  as  in  the  former,  appears  to  me  to  dépend  on  latéral 
sclerosis,  a  trait  common  to  ail  three. 

It  is  not  superfluous  to  remark  that,  when  the  disease  is  far 
advanced,  the  emaciation  may  reach  its  cUmax  ;  the  thenar  and  hypo- 
thenar  eminences  are  entirely  wasted  away,  the  palm  of  the  hand 
is  hollowed  ont,  the  forearm  and  arm  are  reduced  almost  to  a 
skeleton.  Then,  generally,  the  spasmodic  rigidity  becomes  less 
marked,  although  the  members  tend  to  retain  the  habituai  attitude 
which  they  hâve  so  long  preserved. 

Some  patients  bave  the  head  in  a  fixed position,  owing  to  the 
rigidity  of  the  muscles  of  the  neck  ;  they  cannot,  without  effort  and 
pain,  flex  or  extend  it,  or  turn  it  to  the  right  hand  or  to  tlie  left. 

In  one  case,  which  I  recently  observed,  the  muscles  which  raise 
the  lower  maxilla  were  contractured  to  such  a  degree  that  the  mouth 
could  be  opened  only  to  an  extremely  limited  extent. 

As  in  ordinary  progressive  amyotrophy,  the  muscular  emaciation 
is  sometimes  masked,  in  amyotrophic  sclerosis,  by  a  luxuriant  lipo- 
matosis,  which  throws  the  wasted  muscles  into  relief,  as  we  find 
shown,  to  take  one  instance,  by  the  case  reported  by  H.  Otto 
Barth. 

IV. 

The  form  of  amyotrophic  paralysis,  which  we  are  studying,  most 
frequently  first  shows  itself  in  one  of  the  superior  extremities,  then 
it  extends  to  the  other  so  as  soon  to  présent  the  appearance  of  what 
is  called  cervical parajalegia.  Though  the  disease  may  hâve  lasted 
only  four,  five,  or  six  months,  or  a  year  at  most,  the  emaciation  bas 
already  attained  a  degree  which  is  only  seen,  in  protopathic  muscular 
atrophy  at  an  advanced  period,  say  two  or  three  years  after  the 
invasion. 

Things  may  remain  in  this  state  for  two,  six,  or  nine  months, 
rarely  longer.  After  this  delay  the  lower  extremities  are  taken,  in 
their  turn,  and,  as  a  gênerai  rule,  they  become  affected,  as  you  will 
soon  see,  in  a  différent  manner  from  the  upper  limbs. 

a.  At  the  outset  paresis  is  perceived,  hère  likewise,  preceded  and 


200        PAEALTSIS  or  LOWEK  EXTREMITIES. 

accompanied  during  some  time  by  formication  and  numbness 
of  the  member.  But,  hère,  tlie  paresis  présents  this  important 
peculiarity,  namelj  :  it  does  not,  like  the  former,  necessarily 
induce  muscular  atrophy.  The  muscles,  on  the  contrary,  may, 
uutil  the  last  period  of  the  disease,  préserve  a  consistence  and  pro- 
minence  which  shall  form  a  singular  contrast  with  the  state  of  the 
upper  extremities. 

The  paraplegia  présents  this  first  particular  feature,  namely  :  it  is 
not  complicated  with  any  paralysis  of  the  bladder  or  rectum,  and 
there  is  no  tendency  to  the  formation  of  bedsores. 

It  is  also  distinguished,  as  you  will  perceive,  by  other  important 
characters.  Difficulty  of  motion  in  the  lower  extremities  makes 
rapid  progress.  The  patient  feels  his  legs  heavy,  and  hard  to  raise 
from  the  ground.  Soon  he  can  no  longer  walk,  except  when 
helped  by  two  assistants.  Lastly,  it  becomes  impossible  for  him  to 
stand,  and  thus  he  is  nearly  bed-ridden  or  reduced  to  pass  the  day 
seated  in  an  arm-chair.  When  things  hâve  reached  this  stage,  an 
interestiug  phenomenon  has  already  been,  generally  speaking,  more 
or  less  distinctly  exhibited.  I  refer  to  the  temporary  or  permanent 
r'igïdïty,  or,  in  other  words,  to  the  spasmodic  contracture  ofthe 
muscles,  deprived  of  voluntary  motion.  Already,  for  some  time 
back,  the  patient  has  remarked  that,  whilst  in  bed  or  sitting  up,  his 
lower  linibs  stretched  out  or  become  flexed  in  spite  of  himself,  and 
retained  for  some  instants  the  attitude  which  they  had  involuntarily 
assumed.  Extension  is  what  usually  occurs  in  this  kind  of  fit  ;  it 
may  even  go  so  far  as  to  détermine  a  quasi-tetanic  stiffness  which 
makes  the  lower  limbs  resemble  rigid  bars  that  may  be  lifted  ail  of 
a  pièce.  They  are  also,  sometimes,  shaken  by  a  convulsive 
tremulation. 

The  rigidity  becomes  exaggerated  when  the  patient,  aided  by  two 
assistants,  tries  to  rise  and  attempts  to  walk.  Then  the  lower  ex- 
tremities become  exceedingly  rigid  in  extension  and  adduction, 
whilst  the  feet  assume  the  attitude  of  the  varus  equinus  club-foot. 
This  rigidity,  which  is  frequently  extrême,  but  sometimes  only 
slight,^  affecting  ail  the  articulations  of  the  limb  through  the  spas- 
modic action  of  the  muscles,  together  with  the  tremulation,  which 

^  I  cannot  say  wby,  in  certain  cases,  the  rigidity  of  tlie  upper  or  lower 
extremities  should  be  little  marked,  whilst  in  others,  on  the  contrary,  it  is  a 
predominating  phenomenon.  Hitherto,  I  bave  not  found  anything,  in  the- 
anatomo-pathological  conditions  which  could  explain  such  différences. 


LABIO-GLOSSO-LARYNGEAL    PARALYSIS.  20Î 

is^  usuallj,  soon  superadded,  render  standing  and  walking  alike 
impossible. 

Tliat  which,  at  first,  is  but  a  passing  phenomenon^  becoines  after 
a  little  while  transformed  into  a  permanent  symptom.  The  mus- 
cular  rigidity  then  persists^  without  cessation  or  respite,  in  the 
flexors  as  iu  the  extensors,  although  it  prédominâtes  in  the  latter. 
It  is  difficult  to  forcibly  flex  the  extended  members,  and  difficult 
likewise  to  extend  the  flexed  extremities.  Usually,  at  this  period,, 
if  the  extremity  of  the  extended  foot  be  flexed,  by  the  hand,  a 
more  or  less  lasting  tremulation  is  set  up  throughout  the  whole 
limb. 

Thus,  gentlemen,  the  motor  impotence  dépends  less  on  a  weak- 
ening  of  the  innervation  than  on  the  spasmodic  condition  of  the 
muscles.  Muscular  nutrition,  it  may  be  remarked,  still  proceeds,. 
for  a  long  time,  in  the  normal  manner. 

It  is  only  in  the  long  run  that  they  are  seen  to  be  affected  by 
fibrillary  movements  and  to  waste  generally  after  the  manner  of  the 
upper  extremities.  Usually,  when  this  atropby  bas  been  carried  to 
a  certain  extent,  the  rigidity  diminishes  without,  however,  ever 
completely  disappearing. 

The  early  invasion  of  the  lower  extremities  and  the  nature  of  the 
phenomena  affecting  them,  form  a  feature  which  contrasts  witli 
what  we  know  concerning  primary  spinal  amyotrophy  in  which,  you 
recollect,  thèse  members  are  only  invaded  in  the  later  periods. 
They  constitute,  as  it  were,  the  character  of  a  second  period  ;  the 
third  being  marked,  as  we  shall  see,  by  the  appearance  of  bulbar 
phenomena. 

V. 

The  appearance  of  the  later  symptoms  is,  in  some  sort,  an  obli- 
gatory  accompaniment  ;  it  bas  never,  up  to  the  présent,  been  found 
wanting.  Thèse  are  the  phenomena  which,  by  their  union,  com- 
pose the  ST/ndromiis  designated  by  the  term  labio-glosso-laryngeaV 
paralysis.  We  will  only  notice,  in  passing,  this  phase  of  the  dis- 
ease,  for  this  is  a  subject  to  which  we  must  recur  again,  when 
treating  of  paralyses  of  bulbar  origin,  in  particular. 

I  will  mention  the  following  symptoms,  merely  in  order  that  one 
of  the  most  curions  parts  of  the  picture  may  not  be  entirelj 
omitted. 

1°.  Paralysis  of  the  tongue  inducing  difficulty  of  déglutition;^. 


202  CLINICAL    SUMMAEY. 

and  a  difficulty  in  the  articulation  of  words  that  may  issue  in  com- 
plète loss  of  speech.  The  paralysed  tongue^  generally,  soon  présents 
a  certain  degree  of  atrophy  ;  it  is  shrunken,  wrinkled,  and  stirred 
by  vermicular  movements. 

2°.  Paralysis  of  the  vélum  palati  rendering  the  voice  nasal 
(nasonnce),  and  concurring  with  the  lingual  paralysis  to  create  the 
difficulty  of  déglutition. 

3°.  Paralysis  of  the  orbicularis  oris,  the  chief  resuit  of  which 
is  to  al  ter  the  appearance  of  the  features.  Tlie  mouth  is  con- 
considerably  enlarged,  transversely,  on  account  of  the  prédominant 
action  of  the  non-affected  facial  muscles.  The  naso-labial  furrows 
are  deeply  marked.  Thèse  several  symptoms  give  a  lachrymose 
look  to  the  face.  Sometimes,  after  laughing  or  crying  especially, 
the  mouth  long  remains  half-open  in  a  permanent  manner,  and 
allows  a  certain  quantity  of  viscous  saliva  to  flow  continually 
forth. 

4°.  Lastly,  owing  to  the  invasion  of  the  nuclei,  whence  origi- 
nate  the  pneumogastric  neives^  grave  disorders  of  the  respiration  and 
circulation  supervene  and  cause  the  death  of  the  patient,  whose 
strength  has  long  been  diminished  from  insufficient  nutrition. 

I  shall  try,  gentlemen^  to  summarise,  in  a  few  lines,  the  sympto- 
matological  characters  of  amyotropliic  latéral  scier om,  considered 
in  what  may  be  called  its  normal  conditions. 

1°.  Paresis,  without  anœsthesia^  of  the  upper  extremities,  accom- 
panied  by  rapid  emaciation  of  the  muscular  masses  and  often  pre- 
ceded  by  numbness  and  formication.  Spasmodic  rigidity  seizes,  at 
a  given  period,  on  the  paralysed  and  wasted  muscles  and  détermines 
permanent  déformations  by  contracture. 

2°.  The  lower  extremities  are  invaded  in  their  turn.  In  the  first 
instance,  appears  a  paresis,  without  anaesthesia  wliich,  promptly 
advancing,  causes  standing  and  walking  to  be,  in  a  short  time, 
impossible.  To  thèse  symptoms  is  added  a  spasmodic  rigidity 
which,  at  first  intermittent,  next  becomes  permanent  and  sometimes 
complicated  with  Unie  spinal  epilepsy.  The  muscles  of  the  para- 
lysed limbs  only  become  atrophied  in  the  course  of  time,  and  never 
to  the  samc  extent  as  those  of  the  upper  extremities. 

The  bladder  and  rectum  are  not  affected  ;  there  is  no  tendency  to 
the  formation  of  bedsores. 

3°.  A  third  period  is  constituted  by  the  aggravation  of  the 
prcceding  symptoms  and  by  the  appearance  of  bulbar  symptoms. 


PROGNOSIS.       PATHOLOGIOAL   PHYSIOLOGY.  203 

Thèse  three  phases  follow  each  other,  in  a  short  space  of  time. 
Six  months  or  a  year  after  the  invasion  ail  the  symptoms  hâve 
accumulated,  and  become  more  or  iess  strongly  marked.  Death 
supervenes  at  the  end  of  two  or  three  years^  on  an  average,  owing 
to  the  bulbar  symptoms. 

Such  is  the  rule  ;  but  the  chapter  of  anomalies^  it  is  well  under- 
stood,  is  also  in  existence.  Thèse  latter,  however,  are  few  in 
number  and  change  nothing  essential  in  the  picture  which  I  hâve 
just  traced.  Thus,  the  disease,  in  certain  cases,  begins  by  the  lower 
extremities  ;  again,  it  may  be  confined,  at  the  beginning,  either  to 
one  upper,  or  to  one  lower  extremity;  occasionally,  it  remains 
limited,  for  some  time,  to  one  side  of  the  body,  under  a  hémiplégie 
form.  Finally,  in  two  cases,  it  began  by  bulbar  symptoms.  But, 
thèse,  I  repeat,  are  ouly  secondary  modifications.  The  group  of 
characteristic  symptoms  does  not  fail  to  be  soon  formed. 

The  pro(/nosis,  up  to  the  présent,  is  of  the  gloomiest.  There 
does  not  exist,  so  far  as  I  am  aware,  a  single  example  of  a  case 
where,  the  group  of  symptoms  just  described  having  existed,  re- 
covery  followed.  Is  tins  doom  final?  The  future  alone  eau 
clecide. 

YI. 

It  remains  for  me  now,  gentlemen,  to  collate  the  lésions  with  the 
symptoms,  and  to  seek,  in  a  short  essay  of  pathological  j^liysiology, 
the  bond  wliich  unités  them  together. 

1°.  The  paresis  which  appears,  at  the  beginning,  and  the  perma- 
nent contractures  which  after  a  brief  delay  suceeed  it  are,  unques- 
tionably,  dépendent  on  the  symmetrical  and  latéral  scîerosis. 

I  would  remind  you  that,  wherever  latéral  scîerosis  is  found, 
contracture  sooner  or  later  appears  in  a  more  or  Iess  marked  manner. 
Thus,  {a)  in  disseminated  scîerosis  ;  {b)  in  cérébral  hemiplegia  with 
consécutive  dcscending  scîerosis  ;  (c)  in  transverse  myélites  whether 
from  compression  or  spontaneous,  when  latéral  descending  degenera- 
tion  results  ;  {d')  lastly,  in  primary  scîerosis  of  the  latéral  columns 
without  muscular  atrophy. 

2°.  The  paresis  and  contracture  précède  the  atrophy  ;  that  is 
clinically  established.  Hence,  there  is  reason  to  admit  that  the 
latéral  scîerosis,  to  which  they  are  due,  is  produced  before  the  lésion 
of  the  anterior  grey  substance  with  which  the  amyotrophy  is  un- 
questionably  connected. 


204  PATHOLOGICAL    PHYSIOLOGT. 

Througli  wliat  mechanism  does  tlie  lésion  of  the  grey  substance 
become  corabined  with  the  lésion  of  the  white  columns  ? 

Does  it  take  place  through  simple  propagation,  extending  gradu- 
ally  across  the  neuroglia  ? 

It  is  inuch  more  probable  that  the  propagation  is  effected  by 
means  of  the  nerve-filaments  which,  you  are  aware,  normally  esta- 
blish  a  communication  between  the  latéral  columns  and  the  anterior 
cornua.  The  system  of  latéral  columns  tends  to  become  affected 
in  its  entirety  and  very  rapidly.  But  the  lésion  does  not  invade  it, 
throughoutj  at  one  blow.  Thus,  so  far  as  we  can  judge  from  clinical 
révélations,  it  first  of  ail  affects  the  department  which  is  in  physio- 
logical  relation  with  the  movements  of  the  upper  extremities. 
Later  on,  it  reaches  the  department  which  is  connected  with  the 
lower  extremities  ;  lastly,  the  group  of  cerebro-bulbar  fascieuli  is 
invaded  in  its  turn. 

It  is  remarkable  that  the  altérations,  of  which  the  first  and  third 
régions  are  the  seat,  reach  very  rapidly  the  corresponding  parts  of 
the  grey  substance. 

The  muscles  of  the  tongue,  in  fact,  and  those  of  the  upper 
extremities  especially,  begin  to  waste  a  very  short  time  after  the 
appearance  of  paretic  symptoms.  It  is  not  the  same  with  respect 
to  the  System  of  fascicles  relating  to  the  lower  extremities  ;  in  the 
latter  case,  the  paralysis  and  contracture  persist,  for  a  long  time, 
without  the  addition  of  amyotrophy.  Thèse  are  peculiarities  which 
we  can  only  point  out,  without  endeavouring  at  présent  to  offer 
any  plausible  explanation. 


LECTUEE  XIV. 

DEUTEROPATHIC  AMYOTROPHIES  OF  SPINAL  ORIGIN  {CON- 
CLUSION).  HYPERTROPHIC  CERVICAL  PACHYMENINGITIS, 
ETC.,  ETC. 

SuMMARY. — Amyotropliy  connected  îoith  descending  latéral 
sclerosis  consécutive  on  a  circumscribed  lésion  of  the  brain  and 
spinal  cord.     Illustrative  cases. 

Hypertropliic  cervical  meningitis.  Pathological  anatomy  : 
altération  of  tke  méninges;  qf  the  spinal  cord;  of  the  peri- 
pJieral  nerves.  Symptoms  :  painfal  period  {cervical  pjains, 
rigidity  of  nech  ;  formication  and  numhness  ;  paresis  ;  cuta- 
neous  éruptions)  ;  second  period  {paralysis,  atrophy,  hand- 
deformity  {"griffe"),  contracture, patches  of  anœsthesia  ;  para- 
lysis and  contracture  of  the  loioer  extremities) .  Characters 
which  distingidsh  hypertrophie  cervical  pachymeningitis  from 
amyotrophic  latéral  sclerosis. 

Amyotrophies  consécutive  on,  locomotor  ataxia.  Peculiar 
form  of  muscîdar  atrophy  in  suck  cases.     Pathogeny. 

Amyotrophy  consécutive  on  sclerosis  disseminated  in 
patches. 

Subacute  spinal  gênerai  paralysis.  Analogies  with  infantile 
paralysis.     Desideratum. 

Amyot'rop)hies  independent  of  a  lésion  of  the  spinal  cord, 
examples;  pseudo-hypertrophie  paralysis;  saturnine  amyo- 
trophy. 

New  considérations  relative  to  the  tojwgraphie  pathological 
anatomy  of  the  spinal  cord. 

Gentlemen, — To  conclude  tlie  history  of  amyotrophies  of  spinal 
origin,  it  remains  for  me  to  notice  a  certain  number  of  facts  relating 
to  this  subject,  for  which  no  place  could  be  found  in  the  preceding 


206      DESCENDING   LATEEAL    SCLEEOSIS.      AMYOTROPHY. 

lectures.  Tins  task  accomplished,  I  shall  commence  the  study  of 
the  muscular  atrophies  which  dépend  upon  lésions  of  the  bulbus 
rachidicus. 

I. 

At  the  close  of  our  last  conférence  I  endeavoured  to  prove,  by 
taking  clinical  data  as  a  basis,  that^  in  amyotrophic  latéral  sclerosis, 
the  symmetrical  lésion  of  the  latéral  columns,  whence  paralysis  and 
contracture  résulta  is  the  first  to  make  its  appearance  ;  whilst  the 
altération  of  the  anterior  grey  substance^,  with  which  muscular 
atrophy  is  connected^  would  be  a  consécutive  phenomenon.  The 
propagation  of  the  inflammatory  lésion  of  the  white  columns  to  the 
grey  substance  most  probably  takes  place,  I  added,  by  means  of  the 
nerve-tubes  which,  in  the  physiological  state,  establish  a  more  or 
less  direct  communication  between  thèse  two  régions.  Some  of  my 
auditors  hâve,  in  référence  to  this,  made  a  critical  suggestion  which, 
unquestionably,  is  not  without  its  weight.  Why,  they  object,  do 
the  scléroses  (termed  descending) ,  which  are  produced  in  the  latéral 
columns  after  différent  circumscribed  cérébral  or  spinal  lésions,  not 
react,  like  primary  symmetrical  sclerosis,  upon  the  anterior  cornua, 
so  as  likewise  to  induce  the  development  of  muscular  atrophy  in 
the  paralysed  members  ? 

It  is,  in  truth,  a  character  of  the  scléroses  which  supervene  on 
partial  lésions  of  the  brain  and  spinal  cord  that  the  muscles  shall 
remain,  as  a  rule,  free  from  nutritive  disorder,  or,  at  ail  events,  that 
they  shall  only  become  emaciated  in  the  course  of  time,  owing  to 
the  prolonged  functional  inertia  to  which  the  paralysed  members 
are  condemned. 

I  am  not  in  a  position,  gentlemen,  to  résolve  the  difficulty  in  a 
categorical  manner.  I  shall  confine  myself  to  pointing  out  to  you 
that  the  propagation  of  the  lésions  to  the  grey  substance,  in  the  case 
in  question  of  latéral  sclerosis,  is  far  from  being  quite  devoid  of 
précèdent,  and  that  the  muscles  in  the  corresponding  limbs  do,  then, 
suffer  atrophy. 

Thus,  I  hâve  several  times  seen  hemiplegias  of  cérébral  origin, 
(foUowing  on  blood-clot  lésion,  for  instance),  which  were  accom- 
panied,  contrary  to  the  common  rule,  by  more  or  less  marked 
wasting  in  the  paralysed  limbs,  supervening  not  long  after  the 
apoplectic  invasion.  And  in  some  cases  of  this  kind  the  autopsy 
allowed  us  to  ascertain  that  the  anterior  grey  substance — to  which. 


DESCENDING   LATERAL    SCLEROSIS.       AMYOTROPHY.       207 

according  to  tlie  theory  stated,  we  sliould  refer  the  trophic  altérations 
of  the  muscles — participated  in  the  scierons  altération. 

This  fact,  among  others^  has  been  very  distinctly  established  in  a 
case  whose  history  I  reported,  some  time  since,  to  the  Société  de 
Biologie.  The  patient,  a  woman,  aged  70,  had  been  suddenly 
stricken  with  left  hemiplegia,  occasioned,  as  the  autopsy  showed, 
by  the  formation  of  a  hsemorrhagic  focus  in  the  centrum  ovale  of 
the  right  hémisphère.  Contracture  very  rapidly  supervened  in  the 
paralysed  members,  and,  barely  two  months  after  the  attack,  the 
muscles  of  both  the  lower  and  the  upper  extremities  began  to  waste 
away,  exhibiting  at  the  same  time  a  remarkable  diminution  of  elec- 
trical  contractility,  The  muscular  atrophy  made  rapid  progress 
and,  simultaneously,  the  skin  over  every  part  of  the  paralysed 
members,  -wherever  subject  to  the  least  pressure,  became  covered 
with  bullae  and  even  with  eschars. 

An  examination  of  the  spinal  cord  showed  the  présence  of 
descending  sclerosis,  occupying  the  left  si  de  and  presenting  the 
usual  characters  ;  but,  in  addition,  in  several  points  of  the  cervical 
and  lumbar  enlargements,  the  grey  anterior  cornu  on  the  same 
side  offered  the  marks  of  inflammatory  action,  and  in  thèse  points,, 
a  large  number  of  the  great  motor  nerve-cells  had  suffered  very  con- 
sidérable atrophy.i 

Dr.  Hallopeau  has  coUected  at  the  Salpêtrière,  in  the  wards  of 
Professor  Yulpian,  a  certain  number  of  observations  which  are  in 
complète  accordance  with  the  foregoing. 

I  consider  also  that  certain  more  or  less  rapid  muscular  atrophies, 
which  are  produced  in  paralysed  members  in  conséquence  of  trans- 
verse dorsal  myelitis,  are  due  to  the  same  mechanism,  althougli 
the  fact  of  a  lésion  of  the  grey  substance  has  not  yet,  so  far  as  I 
know,  been  verified  de  visu.  I  hâve  described  to  you,  when  speak- 
ing  of  wounds  of  the  spinal  cord,  a  case  which  appears  susceptible 
of  such  an  interprétation. 

Still,  gentlemen,  it  is  the  fact  that  in  consécutive  spinal  sclerosis, 
reaction  on  the  grey  substance  is  the  exception,  whilst,  in  symme- 
trical  sclerosis,  it  is,  as  it  were,  habituai  ;  and  this  is  a  différence  I 
believe,  for  which,  in  the  présent  state  of  things,  no  plausible 
explanation  can  be  supplied.^ 

'  '  Lectures  on  Diseases  of  the  Nervous  System,'  vol.  i,  p.  53. 
^  I  liave  taken  care  to  remark  elsewhere  that  latéral  symmetrical  sclerosis  of 
the  cord  may  exist,  without  participation  of  the  grey  cornua,  and  consequently 


208  CERVICAL  HYPEETEOPHIC    PAOHYMENI^GITIS. 

II. 

But^  I  believe,  I  liave  gone  sufficiently  into  tlie  considération  of 
tliis  question  of  amyotrophic  latéral  sclerosis,  and  it  is,  consequently, 
time  to  begin  the  description  of  some  other  forms  of  denteropathic 
spinal  muscular  atrophj.  That  form,  a  knowledge  of  which  is 
unquestionably  the  most  useful  in  practice,  is  the  one  which 
appears  as  a  complication  of  hypertrophie  cervical  pachymenigitis ,  a 
morbid  state  which  has  engaged  my  attention  for  many  years,i  and 
-which,  quite  recently,  has  been  made  the  subject  of  a  good  mono- 
graphj  by  M.  JofFroy,  one  of  my  students.^ 

This,  gentlemen,  is  clinically  a  rather  well-marked  type;  the 
«ymptomatology  is  usually  distinct  enough  to  allow  a  diagnosis  to 
be  made  without  great  difficulty.  I  will  add,  in  order  the  more  to 
stimulate  your  interest,  that  we  hâve  not  hère  to  deal  with  a  neces- 
sarily  incurable  affection.  At  this  very  moment,  you  can  see,  in 
my  wards,  a  woman  who,  after  presenting  for  five  or  six  years  ail 
the  symptoms  which  characterise  cervical  meningitis,  and  having 
been,  consequently,  long  confined  to  her  bed  in  a  state  of  complète 
powerlessness,  is  now  able  to  walk,  and  also  to  make  use  of  her  upper 
Hmbs  to  do  some  kinds  of  work.  Eecovery  is,  therefore,  possible; 
true,  it  is  almost  always  obtained  with  the  drawback  of  some  infir- 
mities,  which  resuit  from  the  deformities  that  the  disease  almost 
necessarily  induces. 

A.  I  will,  first  of  all,give  you  some  détails  relative  to  the  lésions. 

a.  Hypertrophie  cervical  meningitis,  as  its  name  indicates,  con- 
sists  of  an  altération  of  the  méninges,  more  particularly  afFecting 
the  dura  mater.  As  to  the  position  of  the  lésion  it  is  variable  ;  but, 
it  is  the  cervical  enlargement  of  the  cord  which  appears  to  be,  in 
some  sort,  the  chosen  région.  Altération  of  the  méninges  is  the 
primary  fact,  and  cervical  pachymeningitis  is  that  which  shall  hère 
alone  engage  our  attention  ;  but,  later  on,  the  cord  itself,  on  the 
one  hand,  and,  on  the  other,  the  peripheral  nerves,  which  arise  from 
the  cervico-brachial  enlargement,  are  affected  in  their-turn. 

It  is  probable  that  this  is  not  a  rare  disease.  According  to  ail 
appearance,  the   cases    formerly  published    by  Laennec,    Andral, 

without  being  accompanied  by  muscular  atrophy.  This  is  shown  by,  among 
others,  several  observations  of  Dr.  Westphal  relating  to  cases  of  progressive 
gênerai  paralysis. 

1  '  Société  de  Biologie,'  1871,  p.  75. 

-  A.  Joffroy,  'De  la  pachyméningite  cervicale  hypertrophique  (d'origine 
■spontanée),'  Paris,  1873. 


PATHOLOGICAL    ANATOMY. 


209 


and  Hutin,uncler  tlie  name  of  hypertrophj  ofthe  spinal  cord,  beloDg  to 
cervical  pachjmeningitis.  The  fact  is  that  when  the  racliiclian  canal 
is  opened,  in  a  case  of  this  kind^  one  is  struck  on  seeing  the  cordj 
at  the  brachial  enlargement^  presenting  the  aspect  of  an  elongated, 
fusiform  tumour,  occupying  from  six  to  seven  centimètres  in  height, 
and  voluminous  enough,  consequently,  to  completely  fiU  the  osseous 
canal. 

But,  in  reality,  it  is  not  a  true  hypertrophy  of  the  cord  that  is 
before  the  eye;  for,  on  transverse  sections,  properly  made  (fig.  25), 
it  is  easy  to  perceive  that  the  cord,  far  from  being  hypertrophied  is, 
on  the  contrary,  flattened  from  before  backwards,  and  that  the 
thickened  méninges  are  alone  the  cause  of  the  apparent  augmenta- 
tion in  size  of  the  nerve-column. 


PiG.  25. — Trausverse  section,  taken  from  the  mid  portion  of  the  central  en- 
largement  of  the  spinal  cord,  bj  A.  Castala  (' Pachyméningite  cervical 
hypertrophique/  Thèse  de  M.  Joifroy,  loc.  cit.). 

A.  Hypertrophied  dura  mater,  b.  Nerve-roots  traversing  the  thickened 
méninges,  c.  Pia  mater,  confounded  with  the  dura  mater,  d.  Lésions  of 
chronic  myelitis.  e.  Section  of  two  canals,  newly  excavated  in  the  grey 
substance. 

The  pia  mater  is  also  affected,  but  to  a  much  less  degree  than  the 
dura  mater.  The  latter,  when  closely  examined,  is  seen  to  be 
composed  of  numerous  concentric  layers  (it  may  attain  six  to  seven 
millimètres)  ;  it  is  altered  throughout  its  breadth,  as  is  proved  by 
the  adhésions  which  usually  unité  it,  externally,  to  the  vertébral 
ligament,  and  internally  to  the  pia  mater. 

Sometimes,  the  hypertrophied  paehymeninx  seems  composed  of 
two  layers,  an  external  and  an  internai  one.  The  latter,  which  appears 
to  be  of  new  formation,  is  composed  of  a  dense  fibroid  tissue  ;  it  is 

VOL.   II.  14 


210  SYMPTOMS  :    PAINFUL    PERIOD. 

therefore,  very  distinct  from  those  soft  and  very  vascular  neo-mem- 
branes  which,  in  tlie  spinal  dura  mater,  just  as  in  the  cérébral  dura 
mater,  but  less  frequently  in  the  former  than  in  the  latter,  enjoy 
the  privilège  of  giving  birth  to  hsematomata. 

h.  The  spinal  cord  participâtes  in  the  altération,  and  ail  the  signs 
of  transverse  myelitis  are  found  in  it,  irregularly  disseminated,  and 
attackiug  indifferently  the  central  grey  substance  or  the  white 
columns. 

c.  The  peripheral  nerves  are  attacked,  in  conséquence  of  the 
spinal  lésion,  in  so  far  as  it  bears  upon  their  radicular  course  and 
on  the  anterior  cornua — and  also,  in  their  passage  through  the 
thickened  and  inflamed  méninges.  The  peripheral  nerve  altération, 
generally,  affects  the  anterior  roots  as  well  as  the  posterior — a  circum- 
stance  which  requires  to  be  borne  in  memory  for  the  interprétation 
of  the  symptoms.^ 

B.  The  foregoing  considérations  show  that  the  lésion  is  nowise 
systematic,  and  indicate  the  possibility  of  variations  in  the  clinical 
phenomena.  Nevertheless,  I  repeat,  the  symptomatic  group  is, 
generally,  rather  easy  to  characterise. 

a.  It  is  uot  doubtful  that  the  méninges  are,  first  of  ail,  affected, 
and  soon  afterwards  the  nerve-roots.  The  phenomena  connected 
with  this  double  lésion  constitute  a  first  period,  qx  painful  period, 
which  lasts  two  or  three  months,  the  importance  of  which  cannot 
be  rendered  too  évident. 

In  the  first  place,  we  hâve  extremely  acute  pains,  which  affect 
the  back  of  the  neck,  extending  up  to  the  crown  of  the  head,  and 
spreading  also  to  the  upper  extremities.  Thèse  pains  are  accom- 
panied  by  a  sort  of  figidity,  especially  marked  in  the  neck,  which  is 
kept  immoveabie,  so  as  to  recall  what  we  see  in  sub-occipital  Pott's 
disease.^  They  arewell-nigh  permanent,  but  from  time  totime  exacer- 
bations  are  experienced,  under  the  form  of  paroxysms.  They  react 
frequently  upon  the  joints  which  nevertheless  are  not,  usually,subject 
to  any  swelling.    Concurrently  with  thèse  pains  which,  at  moments, 

'  Participation  of  the  posterior  roots  appears  to  be  a  condition  necessary  to 
the  existence  of  the  symptonis  of  Û\q  painful  period.  This  was  well  shown  iu 
an  observation  recently  presented  to  the  Société  de  Biologie  by  M.  Rendu. 
In  this  case,  which  is  one  of  dorso-lumbar  hypertrophie  pachymeningitis,  the 
posterior  roots  were  spared,  owing  to  the  meningeal  lésions  being  limited  to 
the  anterior  portions  of  the  dura  mater,  and,  as. a  conséquence,  the  painful 
symptoms  were  absent  ('Bulletin  de  la  Société  Anatomique,'  1874,  p.  598). 

2  'Thèse  de  Michaud,'  Paris,  1871. 


PAKALYSIS  AND  ATROPHT.  211 

may  be  extremely  acute,  and  even  horrible,  formications  and  numh- 
nesses  are  noticed  in  the  upper  extremities  which,  at  the  same  time, 
are  affected  by  a  certain  degree  of  paresis.  Lastly,  we  shall  occa- 
sionally  find  pemphigoid  and  hullar  éruptions  afFecting  the  cutaneous 
surface. 

i.  The  preceding  symptoms  appear,  as  you  hâve  understood,  to 
dépend  especially  upon  an  irritation  of  the  peripheral  nerves. 

New  phenomena,  which  constitute  the  second period  of  the  dis- 
ease,  do  no  not  delay  their  appearance;  they  seem  to  dépend, 
particularly,  on  the  extension  of  the  meningeal  lésion  to  the  spinal 
cord,  and  also  ou  a  more  severe  altération  affecting  the  peripheral 
nerves. 

The  limbs  cease  to  be  painful,  but,  on  the  other  hand,  they 
become  paralysed  and  the  muscles  atrophy.  The  wasting  bears 
nearly  equally  upon  the  whole  extent  of  the  limb — a  phenomenon 
which  recalls  that  we  described  when  dealing  with  the  question  of 
amyotrophy  from  latéral  sclerosis.  However,  to  speak  only  of  what 
concerns  the  hand  and  forearm,  it  is  worthy  of  remark  that,  in 
pachymeningitis,  the  muscles  included  within  the  sphère  of  the  in- 
nervation of  the  uluar  and  médian  nerves  are  especially  atrophied, 
whilst  those  which  dépend  on  the  radial  nerve  are  comparatively 
respected.  As  a  resuit  of  this  prépondérance  of  wasting  in  certain 
groups  of  muscles,  we  hâve  a  sort  of  "  claw^''  C^'  griffe")  in  which 
extension  of  the  hand  is  most  marked.  This  griffe  is  not  the  ex- 
clusive appanage  of  cervical  pachymeningitis,  in  which,  indeed,  it  is 
not  constantly  found  ;  but,  as  it  is  not  observed  in  the  other  forms 
of  muscular  atrophy,  it  not  the  less  constitutes  an  interesting  élé- 
ment of  diagnosis,  and  you  know  that,  from  this  point  of  view, 
nothing  should  be  neglected  (fig.  36). 

What  is  the  cause  of  this  comparative  freedom  of  the  dependencies 
of  the  radial  nerve  ?  I  do  not  know.  If  it  were  found  that  the 
originating  filaments  of  the  ulnar  and  médian  nerves  émerge  from 
the  cord  further  down  than  those  of  the  radial,  we  could  thus  un- 
derstand  how  the  latter  miglit,  in  cervical  meniugitis,  remaiu 
outside  the  limits  of  the  morbid  région. 

To  thèse  symptoms  ave  superadded  contractures,  which  seize 
upon  the  paralysed  and  wasted  limbs  ;  and  often  patches  of 
anœsthesia  niake  their  appearance,  on  thèse  extremities,  and  may 
extend  to  the  upper  portion  of  the  trunk. 

This  is  not  aU  :  the  loiver  extremities  become  paralysed  in  their 


212 


CONTRACTURE  OF  LOWER  EXTREMITIES. 


turn,  and  are  afterwards  seized  witli  contracture  just  as  in  primary 
latéral  sclerosis  ;  however,  in  contradiction  to  wliat  we  see  produced 


f  IG.  26. — Attitude  of  the  hand  in  cervical  pacbymeningitis. 

in  ^e  latter  affection,  contracture  of  the  lower  limbs,  in  pachyme- 
ningitis,  does  not  seem  to  be  complicated  with  any  muscular 
atrophy. 

It  does  not  appear  to  me  difïicult  to  supply  the  anatomical  and 
physiological  reason  for  this  paralysis,  and  to  comprehend,  from. 
this  double  point  of  view,  the  absence  of  muscular  atrophy  and  the 
necessary  existence,  so  to  say,  of  the  contracture  in  the  paralysed 
members.  The  motor  paralysis  is  hère  determined  by  the  formation 
of  a  focus  of  transverse  myelitis,  which  is  produced  consecutively 
to  the  meningitis.  The  spasmodic  rigidity  of  the  muscles  dépends 
on  the  descending  sclerosis  which,  consecutively  to  the  transverse 
myelitis,  sooner  or  later  takes  possession  of  the  latéral  white 
columns,  and,  as  in  cases  of  descending  consécutive  sclerosis,  the 
anterior  grcy  cornua  remaining,  as  a  rule,  absolutely  intact,  we 
can  thus  understand  why  the  nutrition  of  the  muscles  is  not  directly 
involved. 

The  constant  absence  of  amyotrophy  is  a  feature  which  already 
distinguishes  the  paraplegia  that  accompanies  amyotrophic  latéral 
sclerosis  from  that  which  is  connected  with  cervical  pacbymeningitis. 
I  would  add  that,  in  the  latter,  we  may  observe  the  production  of 
ansesthesia,  rapidly  developed  bedsores,  and  disorders  of  the  bladder 
and  of  the  rectum,  fiually — a  phenomenon  that  is  absent  from 
amyotrophic  latéral  sclerosis. 


LOCOMOTOR   ATAXIA    AND    AMTOTROPHY.  213 

Many  otlier  distmctive  characters,  taken  in  connection  with 
anatomo-pathological  différences,  would  also  enable  us  to  clinically 
separate  the  two  aÈFections  in  question,  in  spite  of  the  points  of 
resemblance  which  bring  them  together.  Thus,  the  groups  of 
symptoms  which  constitute  what  I  call  the  painftd  period,  the 
partial,  disseminated  ausesthesias,  and  the  bullar  éruptions  are  the 
peculiar  property  of  pachymeningitis  ;  whilst,  on  the  other  hand, 
the  participation  of  the  medulla  oblongata,  which  is  very  rare  in 
the  latter  disease,  appears,  on  the  contrary,  to  be,  as  we  hâve  said, 
one  of  the  necessary  éléments  of  amyotrophic  latéral  sclerosis. 

III. 

Enough  has  been  said  in  référence  to  hypertrophie  meningitis  ; 
at  présent,  I  propose  to  say  a  word  concerning  the  amyotrophies 
tliat  sometimes  supervene  in  the  course  of  locomotor  ataxia  and  of 
duseminated  sclerosis. 

A.  It  is  known  that  progressive  amyotrophy,  more  or  less  gene- 
ralised,  is  not  a  rare  accompaniment  of  posterior  scierons  myelitis. 
To  be  convinced  of  it,  it  will  suffice  to  refer  to  the  numerous  ob- 
servations where  this  coincidence  has  been  noted,  and,  in  particular 
to  those  published  by  MM.  Duménil,^  Virchow,^  Marotte,^  Fried- 
reich,*  Leyden,^  Foucart,^  Laborde,'^  Pierret,^  and  some  others. 
It  results  from  thèse  observations  that,  clinically,  this  muscular 
atrophy  of  ataxic  patients  is  distinguished  by  some  spécial  charac- 
ters.  Thus,  it  does  not  présent  the  regular  mode  of  invasion,  nor 
the  doomed  progressive  course,  peculiar  to  progressive  amyo- 
trophy. Sometimes  disseminated  over  the  most  diverse  parts  of 
the  body,  the  muscular  lésions,  at  other  times,  remain  confined 
to  very  circumscribed  régions,  to  the  foot,  for  instance  (Friedreich), 
to  the  leg  (Leyden),  to  the  back  (Leyden,  Friedreich),  to  the 
nape  (Leyden),  where  they  may  merely  occupy  a  single  muscle, 
or  even  only  a  portion  of  a  muscle.  If  the  thenar  and  hypothenar 
eminences  are  sometimes  affected  (Foucart),  they  remain  perfectiy 

^  Duménil  (de  Rouen),  '  Union  Médicale,'  1862,  No.  17. 
"  'Vircliow's  Archiv,'  Bd.  viii,  Heft  4,  1855. 
2  Marrotte,  '  Union  Médicale,'  11  Juin,  1852. 

■*  rriedreich,  '  Ueber  Degeneratrophie  der  Spinalen  ;'  Hinterstiange, 
■•  Virchow's  Archiv.'  Bd.  xxvi,  xxvii,  1863. 

*  Leyden,  'Die  grauer  Degeuer.,'  Src,  Berlin,  1863. 

6  Toucart,  *  France  Médicale,'  &c.,  Nov.,  1857. 

7  Laborde,  'Soc.  de  Biologie,'  1859. 

^  Pierret,  'Archives  de  Physiologie,'  t.  iii,  1870,  p.  600. 


214 


LOCOMOTOR   ATAXIA   AND   AMYOTEOPHT. 


normal  in  a  large  number  of  cases.  Often  the  muscles  of  the  lower 
extremitieSj  affected  with  motor  incoordination,  are  alone  invaded 
(Laborde,  Duménil).  In  the  case  noted  in  mj  ward,  a  report  o£ 
c 


FiG.  27. — Section  of  the  spinal  cord  in  the  lutnbar  région,  in  a  case  of  ataxia 

complicated  with  muscular  atrophy. 
A.  Sclerosis  of  the  posterior  radicular  zone  ;  left  anterior  cornu  healthy.    d. 

Right  anterior  cornu  atrophied. 

which  bas  been  publisbed  by  M.  Pierret,  the  atropby  affected  the 
whole  extent  of  both  the  upper  and  the  lower  extremities  of  the 
same  side  (fig.  37). 

This  mode  of  distribution  of  the  muscular  lésions  is  already  a  very 
peculiar  phenomenon.  It  should  be  added  that  the  symptoms  of 
posterior  sclerosis,  such  as  the  fulgurant  pains,  ocular  disorder, 
and  motor  incoordination,  &c.,  will  be  always  présent  to  throw 
light  on  the  diagnosis. 

I  hâve  several  times  mentioned  the  mechanism,  according  to 
which,  in  my  opinion,  the  invasion  of  the  anterior  cornuais  effected, 
in  such  cases.  The  irritation  is  propagated  along  the  internai 
radicular  bundles,  whose  course  can  be  foUowed,  anatomically,  to 
the   anterior   grey  substance.^      Quite   receutly,   M.   Hayem  bas 

*  Charcot,  '  Leçons  sur  les  maladies  du  système  nerveux,'  2^  série,  fascic.  i, 
1873,  p.  16.     See  anie,  p.  16. 


DISSEMINATED    SCLEROSIS    AND  AMTOTEOPHY.  215 

given  expérimental  confirmation  to  this  theory.  Avulsion  of  the 
sciatic  nerveSj  in  the  case  of  rabbits,  results  in  a  rent  which  takes 
place  in  the  intra-spinal  course  of  the  posterior  roots.  As  a 
conséquence,  inflammation  is  set  up,  which  extends  along  thèse 
roots  to  the  anterior  grey  cornua,  where  the  groups  of  nerve-cells 
consecutively  undergo  great  altérations^ 

In  ail  known  cases,  the  symptoms  which  attach  to  posterior 
sclerosis  précède  the  development  of  the  amyotrophy.  I  do  not 
believe  that  a  single  example  has  been  published  in  which  the  amyo- 
trophy was,  on  the  contrary,  developed  before  the  tabetic  symptoms. 

B.  I  will  only  make  mention  of  the  muscular  atrophy  which  is 
sometimes  superadded  to  the  ordinary  symptoms  of  disseminated 
sclerosis.  It  supervenes,  according  to  my  observations,  in  cases 
where  the  sclerous  patches  (which,  as  a  rule,  predominate  in  the 
antero-lateral  columns),  invade  the  grey  cornua,  and  there  produce 
profound  altérations. 

IV. 

I  cannot  omit  calling  your  attention  to  another  form  of 
myopathy,  which  M.  Duchenne  (de  Boulogne)  was  the  first 
to  describe,  a  long  time  ago,  under  the  name  of  spinal  gênerai 
paralysïs,  and  which,  speaking  for  myself,  I  as  yet  only  know 
clinically.^ 

This  affection,  up  to  the  présent,  has  not  occupied  the  position 
which  it  rightfuUy  should  take  in  clinical  observation.  Why  is 
this  ?  I  cannot  say,  for  the  facts  of  this  group  are  not  very  rare. 
It  is  an  omission  to  be  regretted.  How  often,  in  fact,  has  not  this 
form  of  amyotrophy,  which  is  almost  always  confounded  with  pro- 
gressive muscular  atrophy,  been  the  occasion  of  errors  in  the 
diagnosis,  and  also,  still  worse,  in  the  prognosis  !  Consulted  on  a 
case  of  this  kind,  and  believing  that  it  is  a  case  of  common  progressive 
amyotrophy,  you  pronounce  a  verdict  of  '^  incurable  "  and,  some 
months  later,  the  patient  may  return  to  you,  completely  cured. 
Could  there  be  anything  better  adapted  to  throw  discrédit  on  the 
physician  ?  Allow  me,  therefore,  to  describe  as  tersely  as  possible, 
the  principal  characters  of  suhacnte  spinal  gênerai  paralysis. 

This  is  a  disease  of  adult  life,  for  it  makes  its  appearance  at  the 

1  Hayem,  "Des  altérations  de  la  moelle,  consécutives  à  l'arrachement  du 
nerf  sciatique  chez  le  lapin  "  ('  Archives  de  Physiologie  '). 

2  'De  rElectrisation  localisée,'  3^  édition,  1872,  p.  458. 


216  SUBACUTE  GENEEAL    SPINAL  PAKALTSIS. 

âge  of  from  35  to  40.  Hereditary  influence  appears  to  count  for 
nothing  in  its  production  ;  but  sometimes,  just  as  in  other  spinal 
affections,  the  influence  of  cold  or  of  a  damp  abode  lias  been 
blamed. 

Spinal  gênerai  paralysis  is  encroaching,  without  being  necessarily 
progressive,  and  recovery, — a  complète  recovery,  as  I  indicated  a 
moment  ago — may  be  hoped  for.  Apart  from  their  mode  of  inva- 
sion and  progress,  the  incidents  of  the  disease  reproduce  rather 
faithfully,  as  you  will  admit,  the  type  of  infantile  paralysis. 

The  paralysis  begins  either  by  the  lower  or  by  the  upper  limbs, 
whence  the  course  is  sometimes  ascending,  sometimes  descending. 
The  motor  inertia  is  accompanied  by  a  well-marked  flaccidness  of 
the  parts  affected. 

Some  passing  formication  excepted,  it  is  not  complicated  with  any 
sensory  disorders.  The  functions  of  the  bladder  and  rectum  are 
respected^  whatever  may  be  the  extent  of  the  paralysis,  and  no  bed- 
sores  are  ever  produced. 

The  atrophy,  which  does  not  long  delay  to  show  itself  in  the 
paralysed  members,  recalls  what  is  observed  in  infantile  paralysis. 
Although  it  prédominâtes  in  certain  muscles  and  groups  of  muscles, 
it  yet  attacks  the  entire  limb,  or  a  whole  région  of  it.  At  the  same 
time,  the  skiu,  over  the  paralysed  and  wasted  parts,  becomes  cold, 
cyanosed,  and  mummified,  as  it  were. 

Lastly,  and  this  is  a  distinctive  feature  which  it  is  important  to 
contrast  with  the  classic  symptoms  of  progressive  muscular  atrophy, 
the  muscles,  even  those  which  hâve  not  undergone  extrême  loss  of 
volume,  présent  a  remarkable  diminution  if  not  a  total  disappearance 
of  faradaic  contractility. 

Thèse  muscles,  which  sufiFer  so  seriously  as  regards  nutrition, 
may  however  occasionally,  as  expérience  demonstrates,  recover  ail 
their  functions,  Nevertheless,  it  is  rare  not  to  find  several  of  the 
most  seriously  stricken  muscles  remaining  atrophied  for  ever. 

It  must  not  be  forgotten  that  a  favorable  prognosis  is  not  the 
absolute  rule.  In  certain  cases,  in  fact,  the  disease  in  its  ascending 
course  may  invade  the  bulbus  and  there  détermine  disorders  analogous 
to  those  which  we  hâve  mentioned  in  connection  with  symmetrical 
latéral  sclerosis  and  which  we  find  again  in  labio-glosso-laryngeal 
paralysis,  properly  so  called.  In  such  circumstances,  the  aspect  of 
things  is  changed,  and  we  may  see  the  disease  shortly  terminating  in 
death. 


PSEUDO-HYPEETEOPHIO    PARALYSIS.  217 

Another  peculiarity  ouglit  to  be  known  to  you  ;  in  its  slow  évo- 
lution^ whicli  sometimes  covers  several  years,  subacute  spinal  gênerai 
paralysis  is  subject  to  illusory  improvements  and  to  relapses.  This 
irregularity  in  the  progress  of  tlie  disease  should  arrest  tlie  attention 
of  the  physician  and  make  him  very  reserved  in  his  judgment. 

I  willj  lastly,  notice  as  a  curions  coincidence — whicli  is  not  rarely 
observed  in  spinal  gênerai  paralysis — namely,  the  occurrence  of 
gastric  disorders^  supervening  in  paroxysms^  which  are  analogous  to 
those  described  under  the  name  of  gastric  crises  in  connection  with 
locomotor  ataxia. 

'  Its  clinical  aspect,  judged  from  what  précèdes,  would  indicate  for 
this  type  an  intermediate  place  between  the  acute  forms  and  the 
chronic  forms  ;  but  pathological  anatomy  lias  not  as  yet  pronounced 
upon  the  question.  It  is  most  probable  that  it  will  contradict  this 
classification  in  nothing.  However,  it  becomes  us  to  wait  for  its 
decree,  before  coming  to  a  definite  conclusion. 

Y. 

I  will  not  delay,  gentlemen,  after  what  has  been  said,  to  enume- 
rate  ail  the  other  possible  forms  of  deuteropathic  spinal  atrophy, — 
that  would  take  us  far  too  long.  The  principles  which  I  hâve  endea- 
voured  to  render  évident  will,  I  think,  suffice  to  guide  you  in  the 
interprétation  of  most  of  the  cases  of  this  kind.  But  I  cannot 
omit  discussing,  at  least  in  a  summary  manner,  certain  amyotrophies 
which  do  not  dépend  upon  a  spinal  lésion,  and  which  are  yet  sus- 
ceptible of  beeoming  generalised,  and  takiug  on  a  progressive  course. 
Among  the  amyotrophies  of  this  group,  I  shall  only  mention,  as 
examples,  the  disease  called  pseudo-hypertrophie  jiaralysls,  and 
saturnine  amyotrophies..  T  am  far  from  wishing  to  enter  particularly 
into  this  question,  I  intend  solely  to  show  that,  as  regards  progres- 
sive amyotrophy,  we  must  take  care  not  to  give  way  to  a  désire  to 
explain  everything,  physiologically,  by  the  lésion  of  the  anterior 
grey  spinal  cornua.  This  lésion  has  its  own  pathogenic  domain, 
which  is  already  very  vast  ;  it  must  not  be  excessively  extended  if 
we  do  not  wish  to  run  the  risk  of  compromising  everything. 

P seudo- hyper trojihic  paralysis,  which  is  also  termed  myo-sclerosic 
is  often  met  with,  as  you  are  aware,  in  young  children  ;  some 
observations,  however,  tend  to  prove  that  it  may  also  show  itself  in 
the  adult. 

However  this  may  be,  considering  the  disease  clinically  we  may 


218  PSEUDO-HYPERTROPHIC   PARALYSIS. 

distinguish  two  periods^  which,  in  fact,  hâve  been  noticed  by  other 
authors.  The  first  period  only  lasts  a  few  months,  or  a  year  at 
most  ;  it  is  characterised  by  a  sort  of  paresis  of  the  lower  extremi- 
ties  especially,  due  to  loss  of  strength  in  the  muscles,  which  as  yet 
show  no  signs  of  seeming  hypertrophy,i  or  which  may  even,  some- 
times,  exhibit  distinct  atrophy.^  In  the  second  period,  which  is  much 
longer,  the  paresis  tends  to  become  generalised,  and  moreover  the 
affected  muscles,  chiefly  those  of  the  calves,  augment  in  size  and 
présent  an  often  enormous  prominence. 

Ail  kinds  of  suppositions  hâve  been  made  in  référence  to  the 
pathogeny  of  the  affection  in  question.  Of  late,  many  authors 
hâve  shown  themselves  inclined  to  look  for  the  starting-point  in 
the  nervous  System,^  and  more  particularly  in  the  spinal  cord. 
This,  in  reality,  is  an  hypothesis  which,  in  my  opinion  at  least,  is 
not  founded  on  any  solid  basis.  Already  we  see,  that  in  a  case 
presented  to  the  Médical  Society  of  Berlin,  by  HH.  Eulenberg 
and  Cohnheim,  a  post-mortem  examination  of  the  nerve  centres 
gave  négative  results.  It  is  true  that  in  this  case,  the  spinal  cord 
having  been  examined  in  the  fresh  state,  or  after  insufficient  hard- 
ening,  very  délicate  lésions,  such  as  atrophy  of  the  motor  nerve- 
cells  and  sclerosis  of  the  anterior  cornua  of  the  grey  substance,  might, 
possibly,  hâve  escaped  scrutiny.'*     But  the  case  which  I  published 

'  Duchenne  (de  Boulogne),  '  Elect.  localisée,'  3*  édition,  p,  605. 

^  Pepper,  '  Clinical  Lectures  on  a  Case  of  Progressive  Muscular  Sclerosis/ 
Philadelphia,  1871,  i)p.  14,  16. 

^  This  opinion  was  formulated  in  an  old  édition  of  Carpenter's  '  Principles 
of  Human  Physiology,'  edited  by  F.  G.  Sniitb,  Philadelphia,  1855,  p.  342, 
note. 

•*  *  Verhandlungen  der  Berliner  Medicinischen  Gesellschafts,'  Berlin,  1866, 
H.  2,  p.  161. 

'"  Charcot,  "  Note  sur  l'état  anatomique  des  muscles  et  de  la  moelle  épiniére 
dans  un  cas  de  paralysie  pseudo-liypertrophique,"  in  '  Archives  de  Physiologie,* 
1871 — 1872,  p.  228. 

The  observation  in  question  relates  to  a  young  patient  attacked  with  pseudo- 
hypertrophie  paralysis,  who  succumbed  at  the  Hôpital  Sainte  Eugénie,  in  the 
wards  of  M.  Bergeron,  in  conséquence  of  an  intercurrent  disease.  The 
history  of  this  little  patient  is  well  known.  It  was  related  by  Dr.  Bergeron  iu 
a  communication  made  to  the  "  Société  Médicale  des  Hôpitaux,"  in  1867 
('  Bulletins  et  Mémoires  de  la  Soc.  Méd.  des  Hôp.  de  Paris,'  t.  iv,  i*^  série, 
1867,  p.  157).  Dr.  Duchenne  (de  Boulogne)  reproduced  it  in  his  raemoir  on 
pseudo-hypertrophie  muscular  paralysis  ('  Archives  Générales  de  Médecine,' 
Janvier,  1868,  et  seq.  p.  19,  ch.  xii).  A  good  photograph  of  the  patient 
standing,  appended  to  Dr.  Bergeron's  paper,  shows  the  exaggerated  promi- 


PSEUDO-HYPERTROPHIC    PARALYSIS.  219- 

twenty  years  ago,  in  the  *  Archives  de  Physiologie  '  is  not  open  to 
the  saine  objections,  and  it  testifies  absolutely  to  the  same  eifect  as 
that  of  M.  Cohnheim. 

nence  presented  by  most  of  the  muscular  masses  in  the  case  of  the  boy  in 
question,  and  enables  us  especially  to  understand  the  characteristic  attitude 
■which  he  assumed  when  standing  (see  also  figs.  3,  4,  and  9,  in  Dr.  Duchenne's 
niemoir).  With  référence  to  the  clinical  aspect  of  the  case,  I  can  do  no  better 
tlian  refer  to  the  works  just  mentioned.  With  respect  to  the  state  of  the 
spinal  cord,  our  observations  were  made  on  transverse  sections,  coloured  with 
carminé,  and  prepared  with  great  skill  by  M.  Pierret.  Thèse  sections  hâve,, 
besides,  been  very  numerous,  and  were  taken  from  différent  parts  of  the  cervical 
and  dorsal  régions  of  the  cord.  I  should  hère  notice  that  the  muscles  which 
receive  their  nerves  from  the  cervical  enlargement  were,  for  the  most  part,, 
affected  to  a  high  degree,  and  that  the  deltoids,  amongst  others,  exhibited 
most  markedly  the  characteristics  of  hypertrophy  by  fatty  substitution.  If 
in  this  case,  the  muscular  lésions  had  been  connected  with  spinal  lésions  the 
latter  should  not  bave  failed  to  show  themselves  well  marked  in  the  cervical 
enlargement  of  the  spinal  cord. 

Now,  the  resuit  was  absolutely  négative.  We  everywhere  found  the  antero- 
lateral  and  posterior  white  columns  in  a  state  of  perfect  integrity  ;  the  grey 
substance,  which  was  the  subject  of  a  very  spécial  investigation,  presented  no 
trace  of  altération.  The  anterior  cornua  were  neither  atrophied  nor  deformed. 
The  neuroglia  retained  its  wonted  transparence,  and  the  motor  cells,  normal 
in  number,  did  not  présent,  in  the  several  parts  which  go  to  constitute  them, 
any  déviation  from  the  normal  type.  Let  us  remember  that  the  spinal  roots, 
both  anterior  and  posterior,  appeared  also  perfeclly  sound. 

Having  recognised  the  fact  that  the  muscular  altérations,  in  this  case,  did 
not  dépend  on  an  altération  of  the  nerve-cells  of  the  anterior  cornua  or  of  the 
nerve-roots,  it  was  important  to  investigate  whether  or  not  they  should  be 
connected  to  some  lésion  of  the  great  sympathetic  or  of  the  peripheral  nerves. 
With  respect  to  the  first  point,  I  can  give  no  information,  the  great  sympa- 
thetic not  having  been  amongst  the  parts  placed  at  my  disposai.  As  regards 
the  second,  I  must  déclare,  after  having  carefully  examined  différent  fragments 
taken  from  the  sciatic,  médian,  and  radial  nerves,  that  thèse  nerves  seemed 
to  me  to  présent,  in  every  part,  the  appearances  of  their  normal  condition^ 
We  even  found,  in  the  substance  of  the  affected  muscles,  several  nerve  fila- 
ments, which  also  appeared  to  us  entirely  free  from  ail  change.  Quite 
recently,  two  most  compétent  observers,  Messrs.  Lockhart  Clarke  and  W. 
Gowers,  presented  to  the  Royal  Society  of  London  a  case  which,  considered 
from  an  anatomo-pathological  point  of  view,  appears  to  be  in  direct  contra- 
diction with  the  foregoing  ("  On  a  Case  of  Pseudo-hypertrophie  Muscular 
Paralysis,"  in  '  Medico-Chirurgical  Transactions,'  vol.  Ivii,  London,  1874). 
But  I  would  point  ont  that,  in  the  observation  of  thèse  English  authors,  the 
spinal  lésions  found  on  autopsy,  and  chiefly  constituted  \i^  foci  of  disintegratioiiy 
only  occupied  in  a  very  accessory,  and  as  it  were,  accidentai  manner,  that 
région  of  the  cord  (anterior  grey  cornua,  and  anterior  radicular  fascicles) 
which  alone  hâve  any  direct  infiueuee  over  the  nutrition  of  the  muscles,  so- 


220  PSEUDO-HYPERTROPHIO    PAEALYSTS. 

According  to  thèse  observations,  pseudo-hypertrophie  paralysis 
ought  to  be  considered  as  independent  of  ail  perceptible  lésion  of 

that  tlie  spinal  lésions,  in  this  case,  seem  to  me  to  bave  been  in  some  sort 
fortuitous.  At  the  very  least,  it  does  not  appear  to  me  they  could  hâve  been 
tlie  starting-point  of  the  markcd  altérations  of  the  muscular  System. 

An  observation  published,  three  years  ago,  in  the  '  Archiv  der  Heilkunde  ' 
("  Beitraege  zur  Kenutniss  der  atrophia  musculorum  lipomatosa,"  Leipzig, 
1871,  p.  120),  by  H.  O.  Barth,  would  also  tend  to  include  pseudo-^i/perirophic 
paralysis  in  the  group  of  spinal  myopathies.  In  fact,  the  autopsy,  made  with 
the  greatest  care,  places  beyond  doubt  the  fact  of  the  existence  of  well-marked 
spinal  lésions  ;  nevertlieless,  I  am  far  from  believing  tliat  this  fact  has  the 
signifieance  attribated  to  it.  The  patient  VFas  a  man,  aged  about  forty-four, 
who  expcrieuced  in  1867,  three  years  bcfore  the  fatal  termination,  the  first 
symptoms  of  motor  paralysis  in  the  lower  limbs.  Two  years  after  the  invasion, 
the  patient  was  confined  to  bed,  and  had  lost  nearly  ail  power  of  motion. 
With  the  progress  of  the  motor  paralysis,  more  or  less  acute  pains,  and  dis- 
agreeable  formicatious  occupied  the  limbs  ;  in  addition,  the  paralysed  muscles 
exhibited  great  atrophy  and  became,  in  certain  parts,  the  seat  of  very  marked 
fibrillary  contractions.  Finally,  the  movements  of  articulation  and  déglutition 
became  difQcuIt. 

In  the  course  of  the  last  months,  several  of  the  atropbied  muscles,  partieu- 
larly  the  adductors  of  the  thumb  and  the  muscles  of  the  calf,  underwent  a 
remarkable  increase  in  size,  although  the  motor  impotence  persisted  to  the 
same  degree. 

At  the  autopsy,  the  muscles  of  the  extremities  mostly  exhibited,  in  différent 
degrees,  the  characters  of  fatty  degeneration. 

As  to  the  muscular  bwidles,  some  of  them  showed  the  altérations  of  simple 
atrophy,  others,  fewer  in  number,  those  of  fatty  granular  degeneration.  In 
the  interval  between  the  bundles,  there  was  a  certain  degree  of  consécutive 
hyperplasia  in  several  points.  An  examination  of  the  spinal  cord  furnished 
some  finteresting  results  :  the  latéral  columns  were  sclerosed,  symmetrically, 
throughout  their  whole  height,  from  the  superior  extremity  of  the  cervical 
enlargement  to  the  inferior  extremity  of  the  lumbar  région. 

The  anterior  cornua  of  the  grey  substance  were  evidently  atrophied;  in 
addition  a  good  number  of  the  large  motor  nerve-cells  presented  more  or  less 
marked  atrophy  and  many  of  them  had  even  disappeared. 

Lastly,  it  was  found  that  a  large  quautity  of  adipose  tissue  had  accumulated 
under  the  skin  of  the  members  and  on  the  surface  of  most  of  the  viscera. 
It  seems  to  me  quite  legitimate  to  refer  the  observation,  the  principal  traits  of 
which  I  hâve  just  summarised,  to  the  classic  type  of  pseudo-hypertrophie 
paralysis. 

The  comparatively  advanced  âge  of  the  patient,  the  existence  of  acute  pains 
and  of  formicatious  in  the  limbs,  the  fibrillary  contractions,  the  difSculty  of 
utterance  and  of  déglutition — ail  thèse  symptoms  would,  if  need  be,  protest 
against  such  an  association.  Ou  the  contrary,  they  very  naturally  couuect 
with  the  morbid  type,  to  which  I  hâve  called  attention  in  the  last  two  lectures, 
and  in  which — as  occurred  in  the  lésion  reported  in  the  observation  by  H.  0. 


INTEGEITY    OF    NERVOUS    SYSTEM.  221 

the  spinal  cord  ;  I  would  add  tliat,  in  my  own  case^  the  nerve-roots 
and  also  the  peripheral  nerves  sliowed  themselves,  as  here^  quite  free 
from  ail  altération.  Hence^  it  is  in  the  muscle  itself  that  we  should 
seek  the  starting-point  cf  the  lésions^  whence  arise  the  symptoms 
observed  during  life. 

In  connection  with  the  muscular  changes  in  pseudo-hypertrophie 
paralysis^  hère  are  some  détails  taken  from  my  work  (loc.cit.),  and 
which  will  doubtless  not  seem  to  you  devoid  of  interest. 

What  first  strikes  the  eye,  in  those  muscles  in  which  it  was  pos- 

Barth — the  symmetrical  sclerosis  of  the  latéral  columns  is  combined  with 
progressive  atrophy  of  the  nerve-cells  of  the  anterior  cornua. 

Undoubtedly,  the  muscular  lésions  described  in  the  case  of  H.  0.  Barth 
recall,  to  some  extent,  those  which  are  uniformly  found  in  ail  cases  of  hyper- 
trophie paralysis  hitherto  published.  But  this  circumstance  would  not,  of 
itself,  be  sufEcient  to  justify  a  nosographic  association.  In  référence  to  this,  I 
thiuk  I  ought  to  make  a  remark  which  might  appear  a  truism,  if  the  fact  to 
which  it  refers  had  not  been  misunderstood.  It  is  this,  namely  :  none  of  the 
muscular  lésions  in  question  are  absolutely  the  peculiar  property  of  pseudo- 
hypertrophie  paralysis,  and  would  not  consequently  suËBce  to  identify  it.  Thus- 
the  hypertrophy  of  the  interstitial  connective  with  simple  atrophy  of  the 
muscjilar  fibres  may  be  met  with,  for  instance,  after  traumatic  lésions  of  the 
nerves  (Mantegazza,  '  Gazetta  Comb.,'  p.  i8i,  1867;  Erb,  '  Deutsch  Archiv,* 
t.  iv,  1868),  and  in  some  cases  of  infantile  paralysis.  (Volkmann,  '  Samral. 
Klin.  Vortraege,'  Leipzig,  1870  ;  Charcot  and  Joffroy,  '  Archives  de  Phys.,' 
t.  iii,  1870,  p.  Ï34).  As  to  the  fatty  substitution  with  or  without  augmenta- 
tion of  size  in  the  muscle,  it  may  also  occur,  as  an  eventual  complication,  iri 
infantile  paralysis  (Laborde,  'Thèse  Inaug.,'  1864;  Prévost,  'Soc.  de 
Biologie,'  1865,  t.  xvii,  p.  213  ;  Charcot  et  Joffroy,  loc.  cit.  ;  Vulpian, 
'Archives  de  Phys.,'  t.  iii,  1870,  p.  316;  W.  Muller,  '  Beitrage  zur  Path. 
Anat.  der  Ruckenmarks,'  Leipzig,  1870,  Obs.  II)  ;  in  progressive  muscular 
atrophy  ;  in  spinal  paralysis  of  the  adult  (Duchenne,  de  Boulogne,  loc.  cit.)  ; 
and  in  many  other  circumstances  which  it  would  take  too  long  to  enumerate. 
It  is  to  be  noticed  that,  in  such  a  case,  the  fatty  substitution  of  the  muscle 
appears  sometimes  to  be  attached  to  a  generalised  lipomatosis,  which  especially 
betrays  itself — as  we  find  exemplifîed  in  Herr  Barth's  case — by  the  accumulation 
of  adipose  tissue  under  the  skin  and  in  the  viscéral  cavities.  Quite  receutly 
H.  W.  Muller  (loc.  cit.)  bas  rightly  insisted  on  this  point.  But  I  dissent 
altogether  from  the  author  just  meutioned  when,  refusing  ail  autonomy  to 
pseudo-hypertrophie  paralysis,  he  maintains  that  ail  the  cases  which  hâve 
been  grouped  under  this  name — artificially  grouped  according  to  him — might 
be  withdrawn  by  a  critical  examination,  and  classified  under  one  of  the 
forms  of  amyotropby  connected  with  atrophy  of  the  motor  nerve-cells.  No- 
thing,  in  my  opinion,  is  less  justifiable  than  this  opinion  ;  and  the  very  case 
which  is  the  principal  subject  of  the  présent  note  would  of  itself  suffice  to 
demonstrate  its  inanity. 


223  LESIONS   OF    MUSCLES. 

sible  to  exactly  stiidy  the  first  phases  of  tlie  morbid  process,  is  that 
the  thin  strips  of  connective  tissue — depending  on  the  perimysmm 
internu7n — which,  in  the  normal  state,  barely  separate  the  ultimate 
muscular  fibres  and  leave  them  almost  in  reciprocal  contact,  are  hère 
replaced  by  thick  septa,  the  short  diameter  of  which,  in  some  places, 
equals  that  of  the  muscular  fibre,  and  even  exceeds  it  (fig.  28). 


fiG.  28. — Transverse  section  of  amuscle,  inpseudo-hvpertrophicparalysis  (in- 
termediate  stage  between  the  first  and  second  period  of  the  morbid  process). 
I,  I.  Islets  of  connective  tissue.  m,  m.  Section  of  muscular  fibres,  g,  g. 
Adipose  cells. 

Thèse  septa,  as  you  may  convince  yourselves  particularly  by  the 
examination  of  dissociated  longitudinal  sections,  are  constituted  by 
connective  tissue  of  récent  formation,  where  the  laminar  fibres, 
especially  disposed  in  a  direction  parallel  to  the  long  axis  of  the 
muscular  fibres,  are  often  iutermingled  with  embryo-plastic  cells, 
rather  numerously  présent. 

The  interposition  of  adipose  cells  between  thèse  fibrillse  marks  a 
new  phase  of  the  process  (fig.  28,  g).  The  cells  are,  at  first, 
scattered,*isolated,  and,  as  it  were,  lost  amid  the  bundles  of  fibrillse  ; 
but  their  uumber  increases,  on  certain  points,  in  such  proportions 
that  they  take  the  place  of  the  fibrils,  which  finally  disappear  com- 
pletely.  This  fatty  substitution,  already  outlined,  as  it  were,  in  some 
places  on  aou-hypertrophied  muscles,  becomes  almost  gênerai  on 
those  in  which  the  increase  of  size  is  much  marked.  In  the  latter 
case,  a  microscopical  examination  shows  the  greater  portion  of  the 
surface  of  the  sections  to  be  occupied  by  fat  cells,  almost  in 
contact  throughout,  heaped  on  the  top  of  each  other  and  rendered 
polyhedrical  by  reciprocal  pressure. 

Hère  aud  there,  amid  the  adipose  tissue^  we  meet — 1°,  either 


LESIONS    OF    MUSCLES.  223 

islets  composed  of  primary  (ultimate)  muscular  fibres  (of  from  i 
to  8,  lo,  or  12  at  most),  entirely  enveloped  bj  connective  fibrillse 
(fig.  28,  1)  ;  or  2°,  isolated  fibril-tracts  without  muscular  fibres  ; 
or  3°^  finally, — and  the  last  is  the  rarest — with  isolated  muscular 
fibres^  deprived  of  tbeir  fibrillary  envelope  and  placed  in  immédiate 
relation  with  tbe  cells  of  the  adipose  tissue  (fig.  29) . 


Fig.  29. — Longitudinal  section  of  a  muscle,  in  pseudo-hypertrophie  paralysis 
(second  period  of  the  morbid  process).  l'at  cells  in  contact  throughout  and 
rendered  polyhedrical  by  pressure.  Isolated  muscular  fibres,  deprived  of 
tbeir  fibrillary  envelolope  and  placed  in  immédiate  relation  with  the  cells  of 
adipose  tissue.  The  muscular  fibres,  even  to  the  smallest  of  them,  hâve 
preserved  their  cross  striation. 

In  short,  the  fatty  substitution  evidently  represents  the  ultimate 
phase  of  the  morbid  process,  and,  according  as  it  advanceSj  the 
new-formed  fibrillary  tissue,  together  with  the  muscular  fibres,  tends 
to  disappear.^ 

1  According  to  Duchenne  de  Boulogne  (loc.  cit.,  p.  603)  and  Poster  ('  The 
Lancet,'  May  8,  1869,  p.  630)  the  apparent  hypertrophy,  observed  in  the  last 
stages  of  the  disease,  would  be  due  to  the  Connective  hyperplasia.  "This  it 
is,"  says  Duchenne,  "which  produces  the  increase  of  volume  in  the 
muscles,  in  direct  ratio  with  the  quantity  of  connective  and  interstitial 
fibroid  tissue  formed  by  hyperplasia.  This  opinion  is  founded  on  the 
results  several  times  obtained  on  examiuing  morsels  of  muscle  extracted, 
during  life,  by  means  of  the  emporte-pièce  histologique.  But,  it  may  be  asked 
whether,  in  this  little  opération,  the  islets  of  tissue  are  not  taken  in  préférence, 
by  the  instrument,  which  would,  on  the  contrary,  find  more  difBculty  in 
laying  hold  on  the  clusters  ot  fat-cells.  It  is  constant  that,  in  cases  where 
fragments  of  hypertrophied  muscles  were  extracted,  during  life,  by  incision, 
thèse  hâve  also  exhibited,  in  a  high  degree,  the  histological  characters  of  fatty 
substitution  (Griesinger  and  Billroth,  Heller  and  Zeuker,  Wernich.  See 
Seidel,    'Die  atrophia  musculorum  lipomatosa,'    Jena,   1867).     The  impres- 


224  LESIONS    OF    MUSCLES. 

As  regards  the  latter,  the  altération  which  issues  in  their  complète 
disappearance  is  already  présent  in  the  first  period,  when  hyperplasia 
of  the  interstitial  connective  tissue  begins,  irrespective  of  any  trace 
of  fatty  substitution.  It  consists  in  a  more  or  less  marked  diminu- 
tion of  the  diameter  ;  many  of  the  fibres  are  so  atrophied  that  it 
requires  the  greatest  attention  to  distinguish  them  in  the  substance 
of  the  interstitial  connective  tissue.  The  greater  portion  of  them, 
however,  even  those  which  hâve  undergone  the  greatest  atrophy, 
préserve  to  the  ultimate  limits  of  emaciation  their  cross  striation 
most  distinctly  évident.  Neither  the  sheath  of  the  sarcolemma, 
nor  the  nuclei  which  it  encloses^  présent  any  altération,  and,  as 
resards  the  muscular  substance,  it  exhibits  no  trace  of  granulo- 
fatty  degeneration. 

You  cannot  fail  to  hâve  been  struck  by  the  analogy  between  the 
muscular  altération  which  has  just  been  described  and  that  which, 
when  viscera  are  in  question,  is  generally  designated  by  the  name  of 
cirrhosis.  Now,  the  lésions  of  muscular  sclerosis  are  observed  in 
very  varions  conditions  ;  and  they  may,  in  particular,  présent 
themselves,  accidentally  it  is  true,  in  différent  forms  of  deuteropa- 
thic  spinal  amyotrophy.  This  circumstance  alone,  namely,  that  the 
invasion  of  adipose  tissue  occurs,  at  a  certain  epoch  of  the  pseudo- 
hypertrophie  paralysis,  as  if  inevitably,  seems  to  me  to  constitute 
a  really  distinctive  character  ;  so  that  the  term  myo-sclewùc  pro- 
posed  by  Duchenne  (de  Boulogne)  should  in  strictness  be  only 
applied  to  the  first  periods  of  the  disease,  whilst  that  of  atrophia 
mîbsculorum  lipomatom  (Seidel),  of  lipomatosa  luxunans  (Heller), 
which  are  somewhat  generally  employed  by  German  authors,  would 
only  apply  to  it  at  an  advanced  period. 

YI. 

The  history  of  pseudo-hypertrophie  paralysis  furnishes  us,  as 

sioE  wbicii  I  bave  received,  after  fréquent  examination  of  the  pièces  which  I 
Lave  studied,  is  that  hyperplasia  of  the  connective  tissue  and  atrophy  of  the 
muscular  fibres  advance,  as  it  were,  by  equal  stages;  the  latter  showing 
itself  ail  the  more  gênerai  and  more  marked  in  proportion  to  the  estent  of  the 
spaces  left  vacant  by  the  atrophy  or  disappearance  of  the  muscular  fibres,  It 
is,  however,  possible  that  the  connective  hyperplasia  may  sometimes  take  the 
upper  hand  and  thus  produce  a  certain  degree  of  seeming  hypertrophy.  But  T 
find  it  difficult  to  comprehend  how  it  could  ever  account  for  the  often  enormous 
increase  of  volume  which  the  muscular  masses  exhibit  at  a  certain  epoch  of 
the  disease,  and  I  am  induced  to  believe  that  substitution  of  fatty  tissue  hère 
plays  the  principal  rôle. 


SATUENINE  AMTOTEOPHT.  225 

you  see,  with  au  example  of  generalised  myopathies,  having  a  pro- 
gressive course,  whicli  develops,  irrespective  of  any  influence  of  the 
nervous  System.  In  the  amyopathles  of  saturnine  ongin,  on  the 
contrary,  the  amyotrophy  appears  to  be  produced  in  conséquence 
of  a  lésion  of  the  peripheral  nerves.  The  existence,  in  such  cir- 
cumstances,  of  an  altération  of  the  nerves  proceeding  to  the  para- 
lysed  and  wasted  muscles,  was  noticed  for  the  first  time,  if  I  mis- 
take  not,  by  M.  Lancereaux.^  This  same  altération  has  been  again 
met  with  in  the  case  of  a  female  patient  in  my  wards,  stricken  with 
lead  palsy,  by  M.  Gombault,  my  clinical  clerk,  who,  in  addition, 
after  a  strict  and  methodical  examination,  ascertained  the  absence 
of  ail  spinal  lésion  in  this  instance." 

The  results  obtained  by  M.  Gombault  bave  been  entirely  con- 
lîrmed  by  a  very  interesting  observation,  recently  published  by  H. 
Westphal.^  Saturnine  muscular  atrophy  seems  therefore  arranged, 
according  to  this,  on  the  same  model  as  ^&  partial  rheumatic  amy 
otrophies,  or  amyoirophies  of  iraumatic  origin,  inasmuch  as  it,  also, 
seems  to  dépend  on  a  lésion  of  the  peripheral  nerves,  and  this 
association  will  appear  ail  the  more  legitimate  because,  in  both 
cases  the  amyotrophy  is  marked,  as  you  are  aware,  by  decrease  or 
even  by  a  more  or  less  rapid  abolition  oîfaradaic  contractUity . 

However  this  may  be,  I  know  not  if  there  at  présent  exists, 
lead-wasting  apart,  a  well-verified  example  of  generalised  amyo- 
trophy arising  from  an  altération  of  the  peripheral  nerves.  I  am 
aware  that,  under  the  name  of  progressive  nervous  atrophy,  the 
description  has  been  given  of  an  affection,  characterised  by 
atrophy  of  progressive  évolution,  arising  from  a  lésion  of  the 
nerves,  without  participation  of  the  spinal  cord.  I  see  no  reason 
which  should  authorise  the  déniai,  a  priori,  of  the  existence  of 
such  an  aflf'ection.'^  But,  I  should  add  that,  at  the  moment,  this 
chapter  of  nosography  seems  to  me  somewhat  to  resemble  a  frame 
without  a  picture.  There  does  not,  in  reality,  exist,  to  my  know- 
ledge,  at  least,  a  single  published  observation  in  which  anatomical 

'  Lancereaux,  'Société  de  Biologie,'  t.  iv,  3e  série,  1862-3,  P-  75- 

-  Gombault,  in  'Archives  de  Physiologie,'  t.  v,  1873,  p.  592. 

^  Westphal,  'Archivf.  Psychiatrie,'  iv  Ed.,  3e  Heft,  1874,  and  'Progrès 
Médicale,'  1874,  ]).  553. 

■^  M,  JofFroy  and  M.  Pierret  hâve  each  recently  communlcated  to  me  a  case 
in  which  a  generalised  atrophy,  rather  ill  characterised  clinically,  would  seem 
to  be  counected  with  lésion  of  the  peripheral  nerves.  The  spinal  cord  was 
quite  healthy  in  both  cases. 

VOL.   II.  15 


226  LESIONS    OP    THE    GREAT  SYMPATHETIO. 

démonstration  lias  been  given  of  this  neuritis,  or  thîs  progressive 
nerve  atrophy,  whence  the  form  of  amyotrophy  in  question  should 
arise.  The  observation  of  M.  Duménil,  interesting  in  its  way, 
wMch  bas  been  relied  on,  in  this  matter,  lias  not  the  significance 
attributed  to  it.  In  this  case,  in  fact,  in  addition  to  the  lésion  of 
the  peripheral  nerves,  there  existed  in  the  spinal  cord  very  serions 
altérations  of  its  central  grey  substance,  and  particularly  of  the 
motor  nerve-cells.  Consequently  we  bave  a  riglit  to  ask  whether 
the  spinal  lésion  may  not  bave  been  the  first  in  date. 

Remarks  of  the  same  kind  may  apply  to  the  cases  published  by 
several  authors,  where  progressive  amyotrophy  is  represented  as  the 
conséquence  of  an  altération  of  the  great  sympathetic.  It  is  not 
doubted  that  lésions  of  the  great  sympathetic,  both  of  the  rami 
communicantes  and  of  the  ganglions,  bave  been  several  times 
observed  in  progressive  muscular  atrophy  ;  but  I  am  not  aware 
that,  in  any  of  thèse  cases,  the  non-existence  of  a  lésion  of  the 
nerve-cells  of  the  anterior  cornua  bas  ever  been  metliodically  esta- 
blished.  On  the  other  hand,  it  is  constant  that  lésions  of  the 
great  sympathetic  are  often  absolutely  déficient  in  the  most  varied 
forms  of  progressive  spinal  amyotrophy.  This  is  peremptorily 
established  by,  amongst  other  proofs,  the  observations  collected 
at  the  Salpêtrière,  by  Dr.  LubimoiF,  of  Moscow,  and  published  in 
tlie  'Archives  de  Physiologie^  (1874). 


VII. 

Hère  terminâtes,  gentlemen,  the  statement  of  the  considérations 
whicb  I  desired  to  lay  before  you  in  référence  to  spinal  amyotro- 
phies.  Ou  our  way,  you  bave  been  able  to  assure  yourselves,  if  I 
do  not  mistake,  that  the  history  of  thèse  affections  bas  been 
placed  in  a  new  light,  owing  to  th€  results  siipplied  by  récent 
researches  relating  to  the  topographie  pathological  anatomy  of  the 
spinal  cord. 

The  particular  ebaracteristic  of  thèse  studies  bas  been,  you 
remember,  to  press  onward,  with  equal  steps,  and  in  close  con- 
nection, clinical  work  and  pathological  anatomy.  It  seems 
opportune  to  show  you,  in  a  brief  summary,  since  the  occasion 
présents  itself,  the  principal  acquisitions  which  are  due  to  thèse 
investigations. 

In  a  gênerai  manner,  they  tend  to  establish  that  the  spinal  cord 


PATHOLOGICAL  TOPOGRAPHY  OP  THE  COED.     227 

is  composecl  of  a  certain  number  of  régions,  answering  in  some  sort 
to  se  many  organs  endowecl  with  spécial  functions.  The  sponta- 
neous,  isolated  lésion,  whether  gênerai  or  partial,  of  eacli  of  thèse 
organs  is  manifested  and  revealed,  during  life,  by  so  many  par- 
ticular  symptomatic  coinpounds,  which  can,  at  présent,  be  corre- 
lated  by  diagnosis  to  their  organic  origin.  Thus  are  constituted,  in 
spinal  pathology,  a  certain  number  of  elementary  affections,  the 
combination  of  which  produces  complex  forms.  The  latter  may,  in 
their  turn,  by  meaus  of  clinical  analysis,  be  decomposed  and 
resolved  into  their  constituent  éléments. 

Expérimentation  had  already,  and  for  some  time,  shown  the  way 
and  had  even  determined  a  certain  number  of  thèse  fundamental 
régions  to  which  I  hâve  just  alluded.  But  it  had  not  at  ail 
advanced  so  far  as  pathology  has  been  able  to  advance  with 
the  assistance  of  the  potent  means  of  anatomical  investigation 
which  we  now  possess. 

I  place  before  you  a  kind  of  topographie  chart,  where  you  will  see, 
indicated  by  différent  tints,  the  several  régions  of  the  spinal  cord 
which  hâve  hitherto  been  explored  by  the  pathologist.  The  ierrœ 
incognitœ  are  left  blank  ;  their  extent,  as  you  perceive,  is  still  great  ; 
but,  it  tends  to  become  less,  day  by  day.  This  is  far  from  being  a 
complète  chart,  or  even  remotely  comparable  to  our  improved  modem 
geographical  maps;  however,  it  is,  perhaps,  a  sketch  superior,  in 
some  respects,  to  the  attempts  of  a  Strabo  or  a  Pomponius  Mêla. 

You  remark  the  old  posterior  columns  decomposed  into  two  very 
distinct  régions  :  i°  GolVs  columns  (fig.  30),  whose  separate  lésion 
has  been  several  times  observed  ;  it  corresponds  to  a  symptomatic 
group,  which  will  doubtless  be  soon  clearly  determined  and  take  its 
place  in  the  clinical  séries;  3°,  the  posterior  radïcular  zones 
(fig.  30,  B,  b),  the  anatomical  substratum  of  progressive  locomotor 
ataxia. 

The  antero-lateral  columns  of  authors  must,  in  their  turn,  be 
decomposed  into  three  régions  :  1°,  the  latéral  columns  joroper, 
A,  A  ;  they  show  themselves  systematically  affected,  throughout  their 
whole  extent,  on  both  sides  of  the  cord,  in  the  case  of  si/mmetrical 
latéral  sclerosis  and,  partially,  on  one  side  of  the  cord  only,  in 
descending  sclerosis,  consécutive  on  circumscribed  cérébral  or  spinal 
lésions  {en foyer). 

2°,  Tiirck's  columns,  a'  ;  their  pathology  is  nearly  always  con- 
founded  with  that  of  the  latéral  columns  ;  3°,  the  anterior  radicular 


328 


PATHOLOGICAL   TOPOGEAPHY   OF    THE    CORD. 


zones,  F;  they  hâve  been  left  blank.     It  lias  beeu,  however,  esta- 
blislied  by  some  observations  that  they  can  be  affected  separately 


V   -  \  '^ 


V 

i 


FiG.  30. — A,  A.  Latéral  columus.  a'.  Tûrck's  coluraus.  b,  Posterior  radicu- 
lar  zones,  c,  c.  Posterior  cornua.  v,  d.  Anterior  coruua.  F.  Anterior 
radicular  zone.    e.  GoU's  columns. 


from  the  rest  (fig.  31^  a).  The  altération  was  représentée!  in  such 
casesj  as  might  hâve  been  foreseen^  by  paralysis  with  amyotrophy 
in  the  member  corresponding  to  the  affected  région  of  the  spinal 
cord. 

With  respect  to  the  grey  substance,  we  know  but  ill  the  effects 
of  an  isolated  lésion  of  the  commissures  ;  and,  as  regards  the  pos- 
terior cornua  (fig.  30  c) ,  we  ouly  know  that,  when  they  are  the  seat 
of  serious  altération,  a  more  or  less  marked  cutaneous  ansesthesia  is 
produced  in  parts  ofthe  bodysituated  on  the  same  side  as  the  spinal 
lésion.  Our  knowledge  is  more  advanced  in  référence  to  the  patho- 
logical  rôle  of  the  anterior  grey  cornua.  It  is,  in  fact,  well  esta- 
bhshed  to-day  that  they  may  be  affected  separately,  primarily  ;  or, 
ou  the  contrary,  in  a  secondavy  manner,  and  it  is  known  that,  in 
botli  cases,  if  the  altération  bears  upon  the  great  motor  cells  the 
production  of  amyotrophy  necessarily  follows. 

The  latter  is  developed  with  rapidity,  if  the  spinal  lésion  be 
evolved  in  an  acute  manner  {infantile  spinal  par alij sis)  ;  or,  on  the 


PATHOLOGICAL  TOPOGRAPHY  OF  THE  CORD.     329 

coutrary^  slowly  and  progressively,  if  its  évolution  be  of  a  chronic 
character  {protojmtàic  spinal  atropJiy  ;  amyotropliic  latéral  sclerosis, 


TiG.  31. — Transverse  section  of  the  lumbar  région  of  tlie  cord,  fron»  a  patient 
tlie  muscles  of  whose  left  lower  extremity  were  paralysed  and  atrophied. 

A.  Left  radicular  zone,  partly  sclerosed.  b.  Right  radicular  zone,  healthy 
c.  The  méninges,  at  this  part,  thickencd  and  inflamcd.  The  corresponding 
anterior  comua  is,  on  the  coutrary,  free  from  altération  (case  communi- 
cated  byM.  Pierret). 

&c).  The  anterior  grey  cornua  [moior  nerve-cells)  and  the  ante- 
rior radicular  zones  (intm-spinal  course  of  the  anterior  roots) 
appear  to  be  the  only  régions  of  the  spinal  cord  which  directly 
affect  the  nutrition  of  the  muscles. 

Such  is  the  state  of  affairs  at  présent.  I  know  not  if  I  delude 
myself,  but  it  seems  to  me  that  the  results  which  hâve  been 
acquired,  ail  imperfect  though  they  are,  allow  us  already  to  anticipate 
a  brilliant  future  for  spinal  pathology. 


PART    FOURTH. 


VARIA. 

SPASMODIC  TABES  DORSALIS.  URINARY  PARAPLEGIAS, 
PARTIAL  MENIÉRE'S  VERTIGO.  POST-HEMIPLEGIC 
CHOREA.     EPILEPSY    OF    SÏPHILITIC  ORIGIN. 


LECTURE  XV. 

ON  SPASMODIC  TABES  DORSALIS. 

SuMMARY. — Provisîonal  dénomination,  ;  Us  justification  ;  symme- 
trical  and prïmary  sderosis  of  tlie  latéral  cohmns.  SjMsmodic 
tabès  dorsalis,  and  ataxic  taies  dorsalis.  Parallel  hetween  the 
two  affections.     Characteristics  of  gait. 

On  contracture  and  trépidation  in  spasmodic  tahes  dorsalis. 
Absence  of  sensory  disorders.  Invasion.  Evolution.  Mode 
of  iiivasion  in  the  limbs.  Prognosis  and  treatrnent.  Biagnosis  : 
disseminated  sderosis  {spinal  fornî),  hysterical  contracture, 
transverse  myelitis,  latéral  amyotrophic  sderosis,  Sfc. 

Gentlemen, — I  shall  to-day  cause  several  patients  to  passbefore 
you,  in  whom  you  can  study  at  your  leisure,  the  symptoms  ot  a 
peculiar  spinal  affection  radically  distinct,  in  my  opinion,  from  ail 
the  other  forms  of  chronic  myelitis  with  which  it  has  been  hitherto 
confounded.  This  affection,  which  I  would  propose  to  designate, 
at  least  provisionally,  by  the  name  of  spasmodic  taies  dorsalis,  is 
not  very  rare,  and  certainly  there  is  not  a  physician  who  has  not 
frequently  met  with  it  in  his  practice.  But,  it  has  not,  I  thiuk, 
been  observed  as  it  deserves.  In  fact,  so  far  as  I  know,  only  one 
author,  Dr.  Erb  (of  Heidelberg),  has  mentioned  it  in  a  spécial 
manner,  and  endeavoured  to  détermine  its  character.  You  will  read 
with  advantage,  in  the  '  Berliner  Klinische  Wochenschrift  '  (No. 
2,6,  1875),  the  short  but  substantial  description  which  he  has  given 
of  it.i 

1  "  Ueber  einen  wenig-bekannteQ  spinalen  symptomencomplex,"  va.  '  Berlin 
Klin.  Wocbenscli./  No.  26,  1875.  Consulfc,  ou  the  same  subject,  an  interesting 
work  recently  published  by  H.  O.  Berger,  of  Breslau  ("  Zur  Pathologie  uncl 
Thérapie  der  Riickenmarkskrankeiten-Primare-Sclerose  der  Seitenstrange," 
Seperatabdruck  ans  der  '  Deutschen  Zeitschrift  fiir  Praktische  Medicin.' 


234  PEOVISIONAL   OHAEAOTER    OF    DENOMINATION. 


It  will  not  be  ont  of  order,  in  commencing.  to  endeavonr  to  justify 
in  a  few  words  the  dénomination^  tliat  may  appear  strange  at  first 
sight,  wliicli  a  moment  ago  I  proposed  we  should  adopt  to  dis- 
tinguish  tliis  pathological  form.  The  affection  in  question  dépends, 
I  concède,  and  this  indeed  is  scarcely  open  to  discussion,  on  an 
organic  suhstratum,  a  more  or  less  grave  anatomical  lésion  situated 
in  the  spinal  cord.  It  is  likewise  certain  that  this  lésion,  to  judge 
merely  from  the  nature  of  the  symptoms,  acts  particularly  on  the 
latéral  spinal  columns. 

It  is  lastly  possible  that,  according  to  a  remark  made  by  Dr.  Erb, 
the  spinal  altération  in  question  may  be  nothing  other  than  the 
systematic  lésion,  first  described  by  L.  Tiirck  and  which  I,  in  my 
turn,  hâve  long  since  made  known  under  the  name  of  primary  and 
symmetrical  scier osis  of  the  latéral  columns  of  the  spinal  cord.  But, 
be  it  remembered,  the  observations  in  which  primary  symmetrical 
latéral  sclerosis,  without  participation  of  the  anterior  grey  cornua/ 
has  been  anatomically  established  and  in  which  clinical  observation 
had,  duriug  life,  revealed  the  existence  of  symptoms  that  to-day 
appear  to  be  attributable  to  the  spasmodic  dorsal  tabetic  group — 
thèse  observations,  1  say,  owing  to  a  curions  concurrence  of  circum- 
stances,  are  ail  of  comparative  old  date. 

They  are  in  some  sort  old  memories,  which  hâve  become  a  little 
indistinct,  and  require  to  be  refreshed.  Hence,  I  think  that  it  will 
be  prudent  to  await  their  vérification  by  autopsies,  before  deciding 
to  name  the  disease  from  any  anatomical  character. 

On  the  other  hand,  the  old  appellation  of  Tahes  dorsalls,  in  spite 
of  the  rather  vague  meaning  always  attached  to  it,  may  serve  well 
enough  to  designate  at  least  a  primarily  chronic  spinal  affection 
which,  like  that  before  us,  advances  almost  as  of  necessity  though 
slowly,  and  which,  after  the  manner  of  locomotor  ataxia  with  which 
it  may  be  paralleled  on  more  than  one  point,  rarely  shows  mercy  to 
its  victim.^     As  to  the  adjective  spasmodic  it  is  employed  to  give 

^  Symmetrical  latéral  sclerosis,  with  participation  of  the  anterior  cornua, 
is  the  anatomical  suhstratum  of  the  verj  distinct  affection  described  by 
Professer  Chareot  under  the  name  of  amyotrophic  latéral  sclerosis  {ante. 
Lectures  XII,  XIII). 

'^  "  Tabcs  Dorsalis  "  (Sauvages,  '  Classis,'  x,  I,  i),  or  "  Dorsualis," 
(Romberg,  '  Lehr.  der  Nervenkrankh.,' Berlin,  1S51,  p.  185).    The  descrip- 


TABES   DOESALIS.  235 

prominence  to  the  dominant  clinical  phenoraenon  ;  I  allude  to  th& 
contracture  which,  almost  from  the  beginning,  occupies  the  affected 
members,  soon  becomes  permanent,  and  in  some  sort  constitutes 
sjmptomatically,  nearly  the  whole  disease.  In  fact,  whilst  pro- 
gressive locomotor  ataxia,  which  might  by  way  of  opposition  be 
called  ataxic  tabès  d  or  salis  as  I  proposed,  first  of  ail  attacks  the 
sensory  spinal  System  (fulgurant  pains,  patches  of  ansesthesia,  and 
of  hypersesthesia)  and  only  secondarily  détermines,  in  the  great 
majority  of  cases,  incoordination  of  the  movements,  spasmodic 
tabès,  on  the  contrary,  remains  confined  through  nearly  the  whole 
duration  of  its  course  to  the  motor  machinery.  It  is  especially 
characterised,  in  short,  during  its  stage  of  complète  development^ 
by  permanent  contracture,  which  gradually  increases  and  sooner  or 
later  renders  the  limbs  powerless,  without  ever  being  accompanied 
by  any  marked  disturbance  of  sensation. 

What  we  hâve  just  said  is  sufficient  to  show  that,  at  présent, 
spasmodic  tabès  has  as  yet  no  real  existence  except  in  the  clinical 
domain,  as  was,  indeed,  long  the  case  with  respect  to  locomotor 
ataxia.  It  is  true  that  it  generally  offers  itself  to  the  clinical  eye 
with  features  marked  enough  to  allow  of  its  being  almost  always 
possible  to  distinguish  it,  in  practice,  not  alone  from  the  great  sys- 
tematic  spinal  disease,  termed  progressive  locomotor  ataxia,  but 
also  from  a7n//ofro2)Mc  latéral  sclerosis,  from  common  transverse 
m,yelitïs,  from  myelliïsfrom  compression,  and  lastly,  though  often 
with  great  difficulty,  from  disseminated  sclerosis,  spinal  fooin.  In 
other  words,  it  may  be  separated  from  ail  the  spinal  affections 
which  in  the  description  given  by  Ollivier  (d'Angers) — a  remark- 
able  one  in  its  way— are  collected  into  a  heterogeneous  group  under 
the  name  oîchronic  myelitis}  and  which  clinical  analysis,  enlightened 
by  pathological  anatomy,  tends  daily  to  disentangle. 

If,  in  fact,  analogies  be  fréquent  amongst  the  several  patho- 
logical states  which  I  hâve  just  enumerated,  differential  charac- 
ters  are  theraselves  not  déficient.  I  hope  to  succeed  in  demonstrating 
this  immediately.  But  I  think  it  opportune,  as  a  preliminary,  to 
ofFer  you  a  description  in  which  I  shall  try  to  depict  prominently 

tioû  given  by  Romberg  refers,  as  is  known,  to  locomotor  ataxia,  "Tabès 
accipitur  commune  ))ro  omni  corporis  ant  partis  extenuatione  "  (B.  Castelli, 
'Lexicon  medicum,  Gen.,'  1745,  art.  "Piithisis  "). 

1  Ollivier  (d'Angers),  '  Traité  des  maladies  de  la  moelle  épiuière,'  3e  edit.^ 
Paris,  1837,  t.  ii,  p.  426. 


•336  AÏAXIC    TABES    DOiiSALIS. 

tlie  dominant  sjmptoms  and  tlie  usual  mode  of  évolution  in  spas- 
modic  tabès.  In  the  course  of  this  description  I  shall,  at  every 
step,  keep  in  view  the  clinical  history  of  locomotor  ataxia,  which 
will  serve  in  some  sort  to  bring  out  the  contrasts. 

I  would  first  of  ail  remind  you  of  the  principal  phenomena  which 
dénote  the  early  stages  of  locomotor  ataxia — I  mean,  of  the  ordinary 
form  of  the  disease — that  which  belongs  to  the  classic  type  created 
by  Duchenne  (de  Boulogne).  In  the  first  rank^  and  long  before 
the  motor  incoordination^  whence  the  disease  gets  its  name,  appears, 
comethe  différent  disorders  of  sensation:  shooting  andpiercing  pains, 
returning  in  paroxysms,  and  afFecting  the  limbs,  the  face,  and  the 
trunk  ;  permanent  fixed  pains  in  certain  points  ;  partial  anaesthesias 
and  hypersesthesias.  The  cephalïc  disorders,  such  as  amblyopia  or 
amaurosis,  and  paralysis  of  the  motor  muscles  of  the  eye,  likewise 
belong  to  this  period.  Lastly,  incontinence  of  urine,  dysuria, 
gastric  crises,  often  testify  already,  in  this  period,  to  the  participa- 
tion of  the  viscéral  nerves. 

Quite  différent,  from  the  begiuning,  is  the  physiognomy  of  spas- 
modic  tabès  dorsalis.  Hère,  the  first  and  for  long  the  only  symptom 
consists  in  a  paretic  condition,  affecting  equally  both  the  lower 
extremities  or  more  marked  in  one  of  them,  and  which  has  at  first 
no  other  effect  than  to  make  walking  rather  difficult,  especially  in 
the  morning  or  evening.  The  patients  depict  the  situation  by 
saying  that  they  grow  quickly  fatigued,  and  that,  wliilst  walking, 
they  clrag  the  leg.  To  that  paresis  is  soon  superadded  a  more  or 
less  marked  tendency  to  muscular  spasms. 

Then,  in  the  horizontal  position,  whilst  in  bed  for  instance,  the 
affected  members  commence  to  stifïen  more  intensely  from  time  to 
time,  in  paroxysms,  especially  in  the  movements  of  extension  and 
adduction.  They  become,  momentarily,  like  rigid  inflexible  bars. 
They  are  often,  besides,  wùthout  visible  cause,  taken  with  a  trépida- 
tion, which  sometimes  remains  limited  to  the  extremities,  and  some- 
times  spreads  over  the  whole  extent  of  the  limb,  and  may  even  affect 
the  entire  body  {spontaneous  treindation).  This  trépidation  may  be 
provoked  by  the  physician  at  will,  as  it  were,  by  abruptly  turning 
up  the  extremity  of  the  foot,  or  toes,  with  the  palm  of  the  hand 
[provoJced  trépidation).  The  rigidity — and  the  same  thing  may  be 
said  of  the  trépidation — becomes  more  striking  when  the  patient 
gets  out  of  bed  and  stands  up.  It  grows  an  ever  greater  hindrance 
to  walking  in  proportion  as,  owing  to  the  progressive  aggravation  of 


ATAXIO  TABES  DOESALIS.  237 

the  disease,  it  grows  more  developed  and  tends  to  become  permanent.^ 
But  uot  till  it  lias  arrived  at  an  advanced  stage,  and  often  after 
many  years,  does  it  definitely  render  walking  quite  impracticable.  I 
confine  myself  to  indicating  thèse  différent  plienomena  without 
entering  into  a  regular  description  because  we  hâve  aiready  studied 
them  at  length  in  connection  with  disseminated  sclerosis,  where 
they  are  found  in  full  détail.^ 

Still,  whatever  may  be  the  intensity  of  thèse  symptoms  affecting 
motion,  sensation  remains  unharmed — no  ansesthesia,  no  hyperses- 
thesia — no  disorders  of  cutaneous  sensibility,  of  whatever  kind,  nor 
of  the  sensibiHty  of  the  parts  situated  beneath  ;  no  lumbar  pains, 
no  girdling  pains,  no  formication,  numbuess,  nor  feeling  of  con- 
striction  in  the  limbs  ;  or,  at  most,  thèse  symptoms,  if  they  exist, 
are  so  shghtly  marked  that  they  must  evidently  be  relegated  to  the 
background  ;  finally,  no  lightning  nor  piercing  pains.  Again,  the 
symptoms,  which  we  term  cephalic,  are,  likewise,  completely  absent  ; 
and  it  is  nearly  the  same  as  regards  disorders  of  bladder  and  rectum 
of  any  note.  Lastly — and  this  is  a  feature  which  it  is  not  unin- 
teresting  to  remark — the  génital  functions  which,  in  the  maie,  are  so 
often  seriously  attacked  when  locomotor  ataxia  is  concerned,  usually 
continue  active,  so  to  speak,  to  the  last,  and  in  nearly  the  normal 
condition,  in  the  case  of  patients  alTected  with  spasmodic  tabès. 

The  démarcation  between  the  two  affections  becomes  probably 
more  striking  still,  in  the  more  advanced  phases  of  their  évolu- 
tion. Usually,  you  are  aware,  the  second  period  of  locomotor 
ataxia  is  made  to  date  from  the  moment  when  the  phenomena  of 
locomotor  ataxia  are  superadded  to  sensory  disorders  and  to  the 
other  symptoms  which  we  just  now  enumerated.  Then  it  is 
that  we  fiud  manifested,  on  the  occurrence  of  voluntary  acts,  those 
contradictory,  disorderly  movements  of  the  lower  extremities,  which 
are  so  exaggerated,  when  the  patient  is  placed  in  the  dark,  as  to 
render  walking  or  standing  very  difficult,  or  even  whoUy  impossible, 
the  peculiar  character  of  which  had  aiready  so  strongly  attracted  the 
attention  of  the  predecessors  of  Duchenne. 

The  locomotor  disorders  become  more  distinctly  defined  in  tabès 

'  When  the  contracture  has  become  permanent  it  exists  even  when  the 
patient  is  in  bed  ;  but  it  shows  itself  more  intense  when  the  patient  attempts 
to  get  up  and  walk.  If  he  be  seated  on  a  somewhat  high  arm-cbair  it  ofteu 
happens  that  his  legs  will  remain  horizontallj  extended,  scarcely  inclined,  and 
that  his  feet  shall  not  touch  the  floor. 

1  '  Lectures  on  Diseases  of  the  Nervous  System,'  and  édition,  vol.  i,  p.  195. 


238  CHAEACTERS    OP    GAIT. 

dorsalis,    as  tlie  tlisease  advances  aud  gets  worse;    but  tliey  are 
characterised  liere  accordiug  to  a  quite  différent  type. 

We  do  not  find  in  tabès  dorsalis  tbose  supple  limbs^  flexible  to 
excess,  appearing  sometimes  as  if  dislocated^  that  unbridled  pro- 
fusion of  movements  whicli  give  to  tbe  walk  of  the  ataxic  patient 
its  characteristic  look  ;  and,  in  conséquence  of  which,  the  feet,  pro- 
jected  forward  and  outvvard,  fall  at  every  step  lieavily  backupon  tlie 
floor,  with  a  stamp.  Hère,  on  the  contrary,  tlie  lovver  extremities, 
rigid  in  every  joint,  energetically  drawn  together,  cannot  separate 
save  after  efforts  in  wliicli  the  pelvic  muscles  seera  to  play  the  chief 
part  and  during  which  the  trunk  is  thrown  backward.  The  feet, 
during  this  time,  are  not  detached,  save  with  great  trouble,  from 
the  floor,  to  which  they  seem  strongly  to  adhère,  producing  as  the 
patient  advances  a  rubbing  or  shuffling  sound,  catcbing  in  the  least 
obstacle,  and  often  becoming  entangled  together.  They  are  fre- 
quently,  in  addition,  stirred  by  the  trépidation  which  may  extend 
to  the  root  of  the  member,  and  may  even,  sometimes,  communicate  to 
the  entire  body  a  kind  of  vibration.  The  patient  advances  in  this 
way,  assisted  by  a  cane  or  by  crutches,  slowly  and  laboriously.  But 
yet  the  gait  is  firm  enough,  and  it  is  a  noticeable  fact  that,  contrary 
to  what  would  occur  in  ataxia,  it  is  nowise  modified  on  closing  the 
eyes. 

This  very  peculiar  gait,  which  I  am  trying  to  depict,  I  hâve 
enabled  you  to  study,  which  is  better,  upon  the  living  model.  It 
had  already  been  remarked  by  Olhvier  (d'Angers),  who  drew  a 
successful  picture  of  it  in  a  passage  which  I  cannot  omit  to  quote  : 

'^Each  foot,''^  lie  says,  "is  withdrawn  with  trouble  from  the 
ground,  and,  in  the  eff'ort  which  the  patient  then  makes  to  lift  it 
entirely  and  to  advance  it,  the  trunk  is  raised  erect  and  thrown 
backward  as  if  to  counterbalance  the  weiglit  of  the  lower  extremity 
which  is  shaken  by  involuntary  trembling  before  it  be  rested  anew 
upon  the  ground.  In  thèse  advancing  movements,  sometimes  the 
point  of  the  foot  is  lowered  and  drags  more  or  less  along  the  ground 
before  being  detached,  sometimes  it  is  lifted  suddenly  whilst,  at  the 
same  time,  the  foot  is  thrown  outward  and  downward.  I  hâve  seen 
some  patients  who  could  not  walk  a  step,  although  assisted  by  a 
cane,  without  throwing  the  body  and  head  backward  so  that  their 
attitude  had  some  analogy  with  that  caused  by  tetanus."  ' 

AU  this  is  perfectly  accurate  and  is  applicable,  in  every  line,  as 
^  Loc.  cit.,  p.  427. 


CHAKACTERS    OF    GAIT.  239 

you  can  verify  for  yourselves,  to  most  of  tlie  patients  wliom  I  liave 
caused  to  défile  before  you.  But,  tliere  exists  one  variety  in  the 
type  whichl  should  mention.  You  can  study  its  characteristics  in 
the  case  of  one  of  tliem,  named  Oss — .  You  see  how,  leanin»- 
on  her  crutches,  this  woman  walks  literally  on  tip-toe,  lier  body 
bent  forward.  This  is  because  at  every  step,  owing  to  the  prédo- 
minance of  the  tonic  spasm  in  the  muscles  of  the  calf,  the  heel  is 
forcibly  lifted,  and  scarcely  touches  the  ground.  Hence,  this  woman^s 
slippers  are  much  worn  at  the  toes.  The  foot,  as  indeed  in  the 
foregoing  cases,  is  taken  with  trépidation  every  time  it  is  ijut  for- 
ward, and,  at  times,  the  trembling  extends  to  the  whole  body. 
When  the  patient  is  going  down  a  slope,  she  feels  as  if  borne  on  by 
the  weight  of  her  body,  obliged  to  quicken  her  step,  and  in  danger 
every  instant  of  falling  upon  her  face.  This  second  mode  of  pro- 
gression, according  to  Dr.  Erb,  would  be  the  most  usual  in  cases  of 
this  nature.  I  am  inclined  to  believe,  judging  from  my  own  obser- 
vation, that  it  is,  on  the  contrary,  less  frequently  met  with  than  the 
first.  Be  this  as  it  may,  we  hâve  now  to  consider  the  two  diseases 
which  we  contrast,  at  the  moment  when  they  hâve  reached  the  last 
term  of  their  évolution.  You  will  be  enabled  to  observe  that  the 
distinctive  characters  are  not  less  marked  in  this  period  than  in  the 
preceding. 

Henceforth,  deprived  of  the  use  of  their  lower  limbs,  incapable 
of  standing  or  walking,  both  the  ataxic  patient  and  the  victim  of 
spasmodic  tabès  pass  the  day  on  a  sofa,  in  an  arm-chair,  or  remain 
confined  to  bed.  But  it  is  easy  to  perceive  that  the  cause  of  the 
impotence  is  radically  différent  in  the  two  cases.  In  the  case  of  the 
ataxic  patient  reduced  to  this  state,  it  is  still  the  deficiency  of  co- 
ordinating  power  which  must  bear  the  blâme.  The  movements 
are  energetic  yet,  even  violent,  and  may  long  remain  so  ;  but  they 
can  no  longer  be  adapted  to  the  exécution  of  physiological  acts.  In 
spasmodic  tabès,  on  the  contrary,  the  motor  impotence  is  evidently 
due  to  the  contracture,  which,  carried  to  an  extrême  and  havin"- 
become  absolutely  permanent,  keeps  the  limbs  invincibly  in  forced 
extension  and  abduction,  thus  rendering  ail  voluntary  movement 
impossible.  On  the  other  hand,  the  spontaneous  or  provoked  tré- 
pidation which,  except  in  the  case  of  some  very  rare  complications 
is  not  observed  at  any  period  in  locomotor  ataxia,  continues  hère 
to  be  produced  to  a  high  degree.^     It  sometimes  even  becomes  so 

^  It  would  be  hard  for  me  to  say  at  what  period  the  phenomenon  of  trepi- 


240  SPONTANEOUS    AND    PKOVOKED   TREPIDATION. 

exaggerated  as  to  détermine  in  tlie  lower  limbs  real  convulsive 
crises,  coming  on  paroxysmally,  of  whicli  tlie  dénomination  spinal 
epilejisy  gives  a  sufficiently  good  idea. 

dation,  so  often  mentioned  in  the  course  of  this  lecture,  was  fîrst  observed 
and  describcd. 

In  an  observation  relating  to  a  woman,  affected  with  disseminated  sclerosis, 
which  we,  Professer  Vulpian  and  myself,  made  in  1862  at  the  Salpêtrière,  we 
referred  to  it  in  tlie  following  terms  :  "  Wlien  one  of  this  woman's  feet  is 
flexed  and  kept  flexed,  by  the  hand  of  an  observer,  there  is  then  produced  a 
trembling,  difficult  to  repress,  and  even  impossible  to  stop  at  times,  when  the 
experiment  is  made  with  the  right  foot."  Hère  we  fiud  clearly  indicated, 
and  that  for  the  first  time,  I  believe,  both  the  epileptoid  tremor  and  the  best 
method  of  inducing  it. 

In  1866,  in  a  note  read  before  the  Société  Médicale  des  Hôpitaux,  M.  Vul- 
pian published  a  complète  history  of  the  case  in  question,  with  the  addition  of 
the  other  observations  which  I  had  communicated  to  him,  and  in  which  the 
phenomenon  was  likewise  mentioned  ('Union  Médicale,'  June,  1866). 

In  this  inaugural  thesis,  composed  under  my  direction,  Dr.  P.  Dubois 
pointed  ont  anew  this  epileptoid  trépidation,  as  showing  itself  at  a  certain 
period  of  disseminated  sclerosis,  of  paraplegias  by  compression,  of  sclerosis  of 
the  cmtero-laieral  columns,  of  chronic  myelitis,  &c.  "  Différent  manœuvres," 
says  the  author,  "  coiitribute  to  originate  it,  such  as  the  application  of  cold  to 
the  skin,  compression  of  the  paralysed  muscles,  energetic  (dorsal)  flexion  of 
the  foot  "  ('  Etude  sur  quelques  points  de  l'ataxie  locomotrice  progressive,' 
Paris,  1868).  I  would  add,  that  in  the  course  of  the  teaching  of  both  Professor 
Vulpian  and  myself,  we  hâve  oft-times  called  the  attention  of  studeuts  to 
this  singular  phenomenon  (Charcot,  '  Leçons  sur  les  maladies  du  Système 
nerveux,'  ire  édition,  t.  i,  p.  218,  1872-73). 

Professor  Brown-Sénuard,  in  his  turn,  made  known  in  ï86S,  in  the  '  Archives 
de  Physiologie,'  an  interesting  peculiarity  relative  to  the  trépidation.  It  was 
known  already  that  the  best  method  of  developing  it  cousisted  in  abruptly 
raising  the  toes,  or  the  whole  anterior  part  of  the  foot.  He  showed  that  the 
inverse  manœuvre,  namely,  plantar  flexion  of  the  toes,  when  abruptly  made^ 
would  détermine  its  cessation. 

Two  years  ago,  Drs.  Erb  and  Westphal  studied  anew  and  with  great  care 
the  epileptoid  trépidation,  under  the  name  of  Eoot-phenomenon  ("  Fuss-phse- 
nomen  :"  W.  Erb,  "  Ueber  Sehnenreflexe  bei  gesunden  und  bei  Riickenmarks- 
kranken.,"  in  'Archiv  fiir  Psychiatrie,'  1874,  p.  792;  C.  Westphal,  "Ueber 
einige  Bewegungs  Erschcinuugen  an  Gelaehmten  Gliodern,"  idem,  p.  803). 
Considering  that  the  percussion  or  excitation  of  the  tendo  patellse  or  of  the 
tendo  Achillis  are  effective  ways  to  develop  this  kind  of  tremor,  thèse  authors 
hâve  sought  to  establish  that  it  bas  its  origin  in  an  excitation  of  thèse  tendons. 
Acoordiug  to  Dr.  Erb,  it  would  be  a  reflex  act,  the  starting-point  of  which 
would  be  an  irritation  of  the  nerves  of  certain  tendons.  The  interprétation 
suggested  by  Dr.  Westphal  is  différent  ;  according  to  him,  the  struck  or 
stretched  tendon  acts  upon  every  part  of  the  corresponding  muscle,  which 
uîJttarr  t&îs  influence  enters  iuto  contraction.    M.  Joffroy  ('  Gazette  Médicale,' 


MODIFICATION   OF   SENSIBILITY.  241 

In  addition  to  thèse  disorders,  tliere  also  exists  in  the  period  which 
vie  are  considering  many  other  pheiiomenacalculated  to  differentiate 
the  two  diseases.  Thus,  in  ataxia,  the  différent  sensory  disturbances, 
so  marked  even  in  the  first  stages,  persist  or  grow  worse.  The  same 
may  be  said  with  respect  to  the  cephalic  symptoms,  and  functional 
disorders  of  the  rectum  and  bladder.  Most  usually,  the  urine  becomes 
fetid,  and  purulent  in  conséquence  of  ulcérons  inflammation  of  the 
vesical  mucous  membrane.  This,  indeed^  is  one  of  the  great  dangers 
which  threaten  the  lives  of  the  patients.  Nothing  of  the  kind 
is  observed  in  the  case  of  persons  attacked  with  spasmodic  tabès. 
Sensibility,  for  instance,  remains  untroubled  in  their  case,  till  the  last 
moment  ;  no  disorders  of  the  sight,  nor  strabismus,  nor,  in  a  word, 
any  of  the  symptoms  which  we  name  cephalic.  The  only  disorder  in 
the  émission  of  urine  which  is  remarked  takes  place  in  the  case 
of  women,  and  chiefly  dépends  on  the  difiiculty  they  expérience  in 
separating  the  thighs.  Let  us  add  that  there  is  no  tendency  to 
the  formation  of  eschars,  and  that  the  muscular  masses  of  the 
lower  extremities  which  in  ataxic  patients  often  become  extremely 
«maciated,  on  the  contrary  préserve,  in  spasmodic  tabès,  a  pro- 
minence  and  consistency  in  some  sort  proportionate  to  the  intensitv 
of  the  spasmodic  contraction  which  holds  them. 

II. 

Enough,  I  think,  has  been  said  to  show,  as  I  had  indicated,  that 
between  progressive  locomotor  ataxia  and  spasmodic  tabès,  the 
contrast  is  striking  on  some  fundamental  points.  Consequently  I 
can  now  leave  the  parallel  aside,  in  order  to  concentrate  your  atten- 

1875,  and  '  Société  de  Biologie  '),  in  criticising  tiiese  works,  bas  defended  the 
opinion  current  till  then,  according  to  which  the  epileptoid  trépidation  would 
be  generally  provoked  by  cutaneous  peripheral  stimulation.  He  combated 
the  opinion  of  Dr.  Erb,  where  it  appeared  to  be  too  absolute.  He  admits, 
with  this  distinguished  pathologist,  that  excitation  of  the  tendons  is  one  of  the 
principal  causes  which  détermines  trépidation,  and  he  does  not  refuse  to  look 
on  this  as  an  example  of  tendon-rejiex  ;  but,  on  the  other  hand,  he  quotes 
cases  where  unquestionably  the  sole  provoking  cause  of  tlie  phenomenon  was 
a  slight  irritation  of  the  skin  (skin-reflex).  As  regards  the  theory  of  Dr. 
Westphal,  it  seems  invalidated  by  the  récent  experiments  of  HH.  Schultze 
and  Farbinge  (' Centralblatt,'  1876). 

I  would  point  out  that  the  phenomenon  of  epileptoid  trépidation  is  not  peculiar 
to  the  lower  limbs.  It  may  be  provoked,  as  I  hâve  long  siuce  demonstrated, 
in  certain  cases  of  hemiplegia,  in  the  coatractured  upper  limb,  by  bending 
back  the  digital  phalanges. 

VOL.   II.  16 


242  SPASMODIO  TABES  :    INVASION,    CAUSES,    ETC. 

tion  on  the  last  of  thèse  affections,  the  description  of  whicli  I  wisli 
to  complète  by  a  few  additional  traits. 

It  appears  to  show  itself,  chiefly  between  the  âges  of  thirty  and 
forty.  I  am  inclined  to  believe  that  it  is  shghtly  less  fréquent  in 
women  than  in  men,  It  is  not  a  very  common  disease  ;  I  hâve  not 
been  able,  after  searching  through  this  vast  hospital,  to  gather 
together  more  than  five  cases  of  the  kind,  while  it  would  hâve  been 
easy  for  me  to  collect  two  score  of  ataxic  patients. 

The  causes  under  the  influence  of  which  it  is  developed  remain, 
up  to  the  présent,  absolutely  unknown  ;  however,  arather  common- 
place  influence,  namely,  the  prolonged  action  of  damp  cold,  is 
found  mentioned  in  several  of  our  observations.  Its  évolution  is 
jirogressive,  but  eminently  slow.  In  the  case  of  the  patients  whom 
I  introduce  to  you,  and  of  whom  several  can  yet  stand  and  walk  in 
a  kind  of  a  way,  its  first  symptoms  date  back  eight,  ten,  and 
fîfteen  years.  It  is  not,  besides,  rare  to  find  that,  after  having 
reached  a  certain  degree,  it  will  remain  stationary  for  several  years. 

Often  cônfined  during  the  whole  of  its  course  to  the  lower  extre- 
mities  which,  as  a  rule,  are  the  first  to  be  attacked,  it  may  extend 
but  always  at  a  late  period  to  the  upper  limbs.  The  latter,  then, 
become  the  scène  of  the  différent  phenomena  which  we  hâve  noticed 
above,  and  which  appear  in  succession.  At  first  it  is  a  paretic 
state  of  the  hands,  which  become  inexpert  in  taking  and  holding 
things.  Prom  time  to  time  the  fingers  are  involuntarily  bent  in 
upon  the  palm  of  the  hand.  Afterwards,  this  pathological  flexion 
becomes  permanent  and  incurable.  Next  cornes  the  turn  of  the 
wrist,  then  that  of  the  elbow,  which  stiffen  in  extension  aud  pro- 
nation. When  this  point  has  been  reached,  the  upper  limbs 
remain  motionless,  rigid,  and  more  or  less  strongly  applied  to  each 
side  of  the  body.  In  the  upper  limbs,  the  trépidation  is  certainly 
always  less  marked  than  in  the  lower  extremities  ;  I  hâve  not  yet 
often  remarked  it  there  in  a  very  distinct  manner.^ 

The  sacro-lumbar  muscular  masses  and  the  muscles  of  the 
abdomen  may  also  be  affected.  Hence  the  belly  is  prominent,  hard 
upon  pressure,  separated  from  the  base  of  the  thorax  by  a  hori- 

^  There  is  one  circumstanee  wliich  is  nearly  absolutely  hostile  to  the  arti- 
ficial  production  of  trépidation;  it  occurs,  namely,  when  the  rigidity  of  the 
limbs  in  extension  is  carried  to  tlie  extrême.  The  manœuvre,  on  the  contrary, 
siiccecds  nearly  always  •when  wo  are  able  to  produce  a  slight  flexion  iu  the 
kuce.joiut. 


COUESE    AND  TEEATMENT.  243 

zontal  furrow,  more  or  less  deep.  At  the  same  time^  tlie  back 
becomes  somewhat  liollowed.  Tliese  phenomena  are  especially  dis- 
ceruible  wlien  the  patients  are  in  bed.  The  exacerbation  which  is, 
at  moments,  produeed  in  the  contraction  of  the  abdominal  muscles 
may  hâve  the  elFect  of  temporarily  determining  a  certain  amount  of 
difficulty  in  the  breathing. 

However,  in  spite  of  the  progress  of  the  disease,  the  gênerai 
health  remains  indefinitely  unshaken.  The  function  of  nutrition 
iu  particular,  even  in  patients  almost  completely  confined  to  bed, 
goes  on  in  the  normal  way,  alike  in  the  motionless  limbs  and 
throughout  the  system  generally.  It  does  not  appear  that  the  dis- 
ease  can,  by  the  fact  of  its  own  symptoms  merely,  ever  directly 
détermine  the  fatal  termination.  The  latter,  undoubtedly,  always 
supervenes  through  the  intervention  of  some  intercurrent  affection. 
One  of  our  patients  has,  for  some  months  back,  exhibited  un- 
equivocal  signs  of  pulmonary  tuberculisation.  I  would  remind  you, 
in  référence  to  this  point  which  I  hâve  already  oft-times  remarked 
upon,  that  this  is  a  rather  fréquent  complication  of  spinal  sclerosis, 
at  an  advanced  period. 

When  spasmodic  tabès  bas  once  been  established,  can  it  ever 
spoutaneously  recède,  or  can  its  course  be  terminated  by  therapeatic 
means  ?  I  cannot  say.  Witli  respect  to  the  latter  point,  the 
attempts  which  I  bave  made,  even  when  the  disease  had  not  reached 
its  utmost  development,  hâve  hitherto  had  been  of  little  effect.  The 
lengthened  employment  of  methodical  hydropathy  which,  incertain 
forms  of  ataxia,  induces  such  liappy  results  ; — the  repeated  appli- 
cation of  the  actual  cautery,  in  points,  along  the  vertébral  column  ; 
— that  of  the  continued  current  bave  not  succeeded  up  to  the 
présent,  so  far  as  my  practice  is  coucerued,  in  doiug  more  than 
effecting  a  temporary  improvement.  The  exhibition  of  the  bro- 
mides  of  potassium,  sodium,  or  ammonium,  M^hether  administered 
in  combination  or  separately,  lias  the  almost  certain  effect  of 
diminishing  or  even  of  completely  putting  an  end  to  the  trépidation 
and  contracture.  But  it  lias  been  always  necessary  to  go  on  to 
very  large  doses  in  order  to  obtain  this  resuit,  which,  besides,  lias 
never  persisted  more  than  a  few  days  after  the  use  of  the  medicine. 
Dr.  Erb  bas  been  more  fortunate  ;  he  has  seen  recovery  take  place, 
in  one  case  whicli,  was  indeed,  of  récent  date,  and,  in  other  cases, 
he  states  that  he  has  obtained  real  and  lasting  improvement  by 
means  of  galvanotherapy. 


244  DIAGNOSIS. 

Hence  we  liave  still  reason  io  liope  that  tlie  prognosis  of  the 
disease  will  become  more  favorable,  when  it  shall  bave  been  better 
studied,  and  when,  above  ail,  observers  sball  bave  learned  to  detect 
it  at  tlie  first  period  of  its  évolution. 

III. 

Clinicallj  to  distinguish  between  spasmodic  tabès  and  loco- 
motor  ataxia,  as  I  trust  to  bave  sufficiently  established  in  the 
preceding  remarks,  is  generally  an  easy  task.  The  difficulties 
of  diagnosis  do  not  lie  on  tins  side  ;  where  they  are,  in  truth,  to  be 
found  is  where  the  other  forms  of  chronic  myelitis  come  to  be  cou- 
sidered.  I  should  wish  to  try  to  show  you,  now,  by  some  examples, 
how  the  solution  of  the  problem  may  most  frequently  be  accom- 
plished.  In  the  first  place,  I  would  hâve  you  remark  that  not  a 
single  symptom  of  spasmodic  tabès  is  really  peculiar  to  itself.  Per- 
manent contracture,  preceded  by  paresis  and  trépidation,  may  in  fact, 
as  the  history  of  hysteria  shows,  be  produced  without  any  trace 
of  spinal  lésion  existing,  so  far  at  least  as  our  means  of  inves- 
tigation can  detect.  The  selfsame  phenomena  are,  on  the  other 
hand,  as  you  know  from  our  preceding  studies,  the  obligatory 
accompaniments,  as  it  were,  of  ail  scierons  inflammations  of  the 
spinal  cord,  whatever  their  origin  may  be,  provided  only  they  affect 
the  latéral  columns  to  a  certain  extent  ;  whether  we  hâve  to  deal 
with  the  insular  form  or,  on  the  contrary,  with  ihQ  fasciculated  form 
of  spinal  sclerosis.  Hence  it  is  that  you  see  them  figure  in  the 
symptomatology  of  very  difl'erent  affections,  in  which  the  columns 
in  question  are  engaged  ;  lasting  hémiplégie  contractures,  consécu- 
tive on  a  circumscribed  cérébral  lésion  {en foyer),  progressive  gênerai 
paralysis,  transverse  partial  myelitis,  whether  primary  or  caused  by 
compression,  amyotrophic  latéral  sclerosis,  sclerosis  in  disseminated 
patches,  &c.  In  the  diagnosis  of  spasmodic  tabès,  therefore,  our 
considération  must  be  given  far  less  to  the  symptoms  themselves 
than  to  their  mode  of  distribution  and  évolution  ;  their  isolation,  espe- 
cially,  whence  results  the  monotony  of  the  clinical  picture  so  peculiar 
to  the  disease,  ought  to  be  regarded  as  an  élément  of  the  first  im- 
portance. For,  it  may  be  asserted  that,  saving  some  fortuitous  com- 
plication, every  spinal  affection,  in  which  sensory  or  mental  disorders, 
functional  disturbance  of  the  bladder  or  rectum,  paralysis  of  tlie 
oculi-motores,trophic  muscular  lésions,  &c.,  are  shown,  in  association 
with  contracture,  is  not  an  example  of  spasmodic  dorsal  tabès.  This 


CHEONIC  TEANSVEESE    MTELITIS.  245 

establislied,  I  will  put  aside  certain  morbid  states,  as,  for  instance, 
hystericaP  and  hémiplégie  contractures,  tlie  differentiation  of  which 
offers  no  serious  difficulty,  in  order  only  to  dwell  upon  tlie  diseases 
which  might  soinetimes  mislead  even  a  practised  eye. 

I  hâve  caused  to  be  placed  before  you  the  woman  Seb — ,  aged 
about  forty,  confined  to  bed  for  the  past  two  years,  having  become 
incapable  of  walking  or  even  of  standing.  You  may  remark  that 
her  lower  limbs,  to  which  it  is  impossible  for  her  to  give  the  least 
voluntary  movement,  are  not  flaccid  and  inert,  as  happens  in  certain 
forms  of  paralysis  ;  on  the  contrary,  they  are  rigid  in  extension  and 
adduction  ;  as  much  résistance  is  felt,  when  you  wish  to  flex  them, 
as  you  would  feel  in  extending  them,  had  they  been  previously 
flexed.  Trépidation  is  provoked  at  will  when  you  turn  up  the  point 
of  the  foot,  and  it  often  occurs  spontaneously  under  the  form  of 
spinal  epilepsy.  I  hâve  known  this  patient  for  many  years  able 
still  to  make  some  laborious  steps  in  the  ward,  leaning  on  the  bed- 
rails  or  pushing  a  chair  before  her,  with  a  firm  grasp  on  its  back, 
and  making  it  slide  along  the  waxed  boards  ;  at  every  step,  her 
trunk  was  strongly  thrown  back,  at  the  same  time  lurching  alter- 
nately  to  one  side  and  then  to  the  other.  In  short,  her  gait  was 
then  nearly  what  I  hâve  just  endeavoured  to  depict  in  my  descrip- 
tion of  spasmodic  tabès.  If  the  examination  were  carried  no 
further  you  might  imagine  that  this  is  an  example  of  that  affection. 
In  order  to  undeceive  you,  it  will  be  enough  to  emphasise  some 
détails  revealed  by  a  less  superficial  scrutiny. 

One  day,  eight  years  ago,  after  having  been  much  fatigued,  she 
remained  for  some  time  in  a  draught  of  very  cool  air,  whilst  her 
person  was  still  in  a  state  of  perspiration.  Shortly  after,  she  expe- 
rienced  a  sensation  which  she  compares  to  that  which  might  be  caused 
by  a  stream  of  iced  water  poured  down  along  her  back.  This  sen- 
sation was  soon  followed  by  a  rather  acute  pain,  accompanied  by  a 
feeling  of  constriction,  and  occupying,  at  the  same  time,  both  back 
and  loins.  The  pain,  which  extends  like  a  belt  on  either  side 
of  the  thorax,  persists  at  présent.  At  the  same  time,  there 
supervened  prickling  and  formication  in  the  lower  extremities.  It 
seems  to  her  as  if  now  a  stream  of  very  hot  water,  and  again  a  stream 
of  very   cold   water,    were   passing  through  them,  that  they  are 

1  On  the  diagnosis  of  hysterical  contracture,  see  Charcot,  '  Leçons  sur  les 
Maladies  du  Système  nerveux,'  t.  i,  p.  347  et  seq.  ;  Bourneville  et  Voulet, 
*De  la  contracture  hystérique.' 


246  LATERAL  AMYOTROPHIC    SCLEROSIS. 

at  moments  bound  by  very  tiglit  cords.  Thèse  phenomena  liave 
never  since  ceased  to  exist. 

Some  days  after  the  appearance  of  thèse  sensory  disorders  paresis 
supervened,  to  which  soon  were  superadded  first  temporary,  then 
permanent  rigidity,  trépidation,  and  fits  of  spinal  epilepsy. 

At  the  présent  moment  you  can  ascertain,  as  we  hâve  oft-times 
found  during  her  five  years  of  hospital  life,  the  existence  of  very 
marked  obnubilation  of  sensibility  of  every  kind  over  the  whole 
extent  of  the  lower  limbs  and  abdomen.  When  you  pinch  the  skin 
of  the  legs  or  thighs,  or  tickle  the  soles  of  the  feet,  you  also  find 
muscular  tremor  produced  by  reflex  action,  and  various  phenomena 
of  dysœstliesia  to  which  I  called  your  attention  while  speaking  of 
paraplegias  consécutive  on  slow  compression  of  the  spinal  cord.^  I 
should  add,  in  order  to  complète  the  picture,  that,  from  the  com- 
mencement, the  need  of  raicturition  liad  become  imperious,  and 
required  to  be  promptly  satisfied  ;  that,  for  many  years  back,  vesical 
paresis  lias  supervened,  necessitating  from  time  to  time  the  use  of 
the  cathéter;  that,  finally,  the  urine  is  usually  fetid,  turbid, 
and  constantly  leaves  a  more  or  less  abundant  muco-purulent 
sédiment. 

The  différent  symptoms,  which  hâve  just  been  reviewed,  tlieir 
mode  of  succession  and  concaténation — in  a  word,  ail  things  com- 
bine, as  you  hâve  understood,  to  establish  that  the  spinal  lésion  with 
which  our  patient  is  affected  is  nothing  else  than  chronic  dorsal 
transverse  mijelïtis,  with  consécutive  descending  scierons  degenera- 
tion  of  the  latéral  columns.  It  seems  to  me  useless  to  insist,  in 
order  to  render  prominent  the  fact  that  the  various  sensory  dis- 
orders, enumerated  above,  that  vesical  paresis  and  the  existence  of 
muco-purulent  urine,  should  be  especially  counted  among  the  sym- 
ptoms adapted  to  deepen  the  démarcation  between  this  form  of 
myelitis  and  spasmodic  tabès. 

In  cases  where  partial  myelitis  is  connected  with  slow  compression 
of  the  spinal  cord,  it  would  be  characterised  in  addition,  in  the 
absence  of  vertébral  déformations,  by  the  existence  of  i\\Q%ç, pseudo- 
neuralgias,  the  clinical  importance  of  which  I  strove,  on  another 
occasion,  to  demonstrate,  and  the  interest  of  which,  in  the  category 
we  are  considering,  would  be  ail  the  greater,  seeing  tliat  thcse  pains 
are  already  exhibited,  as  préludes,  even  before  the  paretic  symptoms 
began  to  appear.  If,  instead  of  comprising  the  whole  breadth  of 
^  '  Lectures  ou  Diseases  of  the  Nervous  System,'  vol.  ii,  p.  99. 


DISSEMINATED   SCLEROSIS,    SPINAL    FORM.  247 

the  cord  a  cross,  the  lésion,  whatever  it  be,  remained  confined  to  a 
latéral  lialf  of  the  nerve  column,  the  symptomatology  would  présent 
itself  under  the  type  of  spinal  hemijjaraplegia  witli  crossed  anœsthe- 
sia,^  and  the  diagnosis  would  be  thereby  rendered  more  easy  of 
détermination. 

Awyotrophic  latéral  sderosis  has  thèse  symptoms  in  common  with 
spasmodic  tabès,  namely  :  paresis  followed  by  rigidity  of  the  limbs, 
absence  of  sensory  disorders,  absence  of  disturbance  of  functions  of 
bladder  and  rectum  ;  but,  it  is  deepîy  distinguished  from  it,  as  it  were 
from  the  beginning,  by  the  atrophy  which  the  muscular  masses  un- 
dergo  in  the  affected  limbs,  by  the  more  rapid  évolution  of  the  disease 
taken  as  a  whole,  and  by  the  regular  appearance,  so  to  speak,  of  the 
bulbar  symptoms,  in  the  last  period.  Besides,  in  this  affection,  it 
is,  as  a  rule,  the  upper  extremities  which  are  first  invaded,  contrary 
to  what  takes  place  in  spasmodic  tabes.^ 

It  remains,  for  me,  in  the  last  place,  to  point  out  the  clinical 
relationships  which,  in  certain  cases,  may  exist  between  spasmodic 
dorsal  tabès  and  disseminated  sderosis  of  the  nerve  centres.  Hère 
it  is,  as  I  hâve  already  indicated  to  you,  that  you  must  expect  to 
meet,  more  than  once,  with  a  stumbling-block  to  your  diagnosis. 
When  multilocular  sderosis  présents  itself,  with  ail  its  singular 
apparatus  of  spinal,  bulbar,  and  cérébral  symptoms,  it  is  certainly 
not  difficult,  in  gênerai,  to  establish  its  identity;  but,  when  we 
hâve  to  deal  with  imperfect  forms — abortive  forms,  as  they  are  still 
called — it  becomes  a  différent  question.  In  fact,  there  is  not  a 
single  pièce  of  the  symptomatic  apparatus  mentioned,  if  I  might  so 
speak,  which  may  not  occasionally  be  déficient.  Thus,  to  give  but 
one  instance,  the  clinical  représentation  of  disseminated  sderosis  is 
found,  in  some  cases,  to  be  reduced  almost  to  mère  contracture  of  the 
lower  limbs,  with  or  without  concomitant  rigidity  of  the  upper  ex- 
tremities {spMalform  of  disseminated  sderosis).^  Even  in  such  a 
case,  the  présent  or  past  coexistence  of  some  of  the  cephalic  sym- 
ptoms, such  as  nystagmus,  diplopia,  peculiar  difficulty  of  utter- 
ance,  vertigo,  apoplectiform  attacks,  spécial  disorders  of  the  mind — 

^  Loc.  cit.,  p.  loi. 

-  Loc.  cit.  p.  202. 

2  Many  cases  of  this  kind  are  coUected  in  the  memoir  presented  by  M . 
Vulpian  to  the  Société  des  Hôpitaux  ('  Union  Médicale/  1865).  M.  Charcot 
has  coUected  some  others  siuce  then  (see  'Lectures  on  Diseases  of  the 
Nervous  System,'  vol.  i,  p.  214). 


248  DISSEMINATED    SCLEROSIS,    SPINAL    EORM. 

this  coexistence,  I  say,  would  furnish  évidence  of  almost  conclusive 
worth.  But,  external  to  this  combination,  I  no  longer  see  on  what 
solid  basis  tlie  diagnosis  may  be  grounded;  there  would  remain 
little  resource  but  what  we  could  draw  frompresuraption.  Perhaps, 
a  more  attentive  and  minute  examination  may  some  day  enable  us 
to  note,  either  in  the  symptomatology  itself,  or  in  the  etiological 
conditions  which  liave  been  so  little  studied  as  y  et,  some  new  traits 
which  hâve  hitherto  escaped  observation,  and  which  will  henceforth 
allow  a  line  of  démarcation  to  be  drawn  between  the  two  diseases, 
at  every  epoch.  The  future  will  reveal  whether  or  not  our  hopes 
in  this  respect  are  illusory.^ 

^  One  of  the  patients  exhibited  at  this  lecture,  who  presented  an  example 
of  spasmodic  dorsal  tabès,  lias  siuce  succumbed.  ïbe  autopsy  bas  not  confirmed 
the  diagnosis,  wbicli,  indeed,  was  given  with  some  qualifications.  It  revealed 
the  existence  of  disserainated  sclerous  patcbes,  limited  to  tbe  lower  portions 
of  the  crus  cerebri,  to  the  anterior  pyramids  in  the  medulla  oblongata,  and, 
in  certain  points,  occiipying  the  posterior  columns  (cervical  région)  ;  in  other 
points  the  latéral  columns  (lower  dorsal  région).  The  cérébral  hémisphères 
were  quite  free  from  altérations  tbroughout.  The  détails  of  this  case  (the 
fourth  in  the  thesis  of  M.  Bétous)  will  be  published  in  extenso  on  another 
occasion.  We  will  tben  be  able  to  notice,  in  the  history  of  the  patient,  the 
existence  of  cervical  and  dorso-lumbar  pains,  vertigo,  an  increase  of  the 
paresis  of  the  limbs  in  darkness,  and  varions  other  circumstances  which  ought 
to  bave  shown  the  way  to  a  right  diagnosis,  had  more  attention  been  given. 
Just  at  présent,  I  wish  to  confine  my  remarks  to  pointing  out  the  difficulties 
which  the  clinical  observer  may  meet  when  be  finds  bimself  coufrouted  witb 
certain  abortive  forms  of  disseminated  sclerosis. 


LECTURE   XVr. 

URINARY  PARAPLEGIAS.i 

SuMMARY. — Preamhle.  Theoreticcd  point  of  view.  Clhùcal  reality 
qf  iirinary  paraplegias.  Définition.  Classification  of  cases 
into  th'ee  groîcps. 

Myelitis  consécutive  on  diseuses  of  the  urinary  passages. 
Tiare  in  women  ;  fréquent  in  men.  Conditions  of  develojjnient: 
gonorrhœa,  stricture  ofthe  urethra,  cystitis,  nephritis  ;  prostatic 
affections  ;  calculons pyelo-nepJiritis.  Exacerbation  of  the  dis- 
ease  of  tJie  urinary  passages  précèdes  the  invasioti  of  spi}ial 
phenomena.  Symptoms  :  formication  ;  anasthesia,  dorso- 
lumbar  and  girdling  pains.  Paraplegia  with  fiaccidness  ; 
excitation,  tlien  abolition  of  reflex  excitability  ;  permanent 
contracture  ;  bedsores.  Position  and  nature  of  the  lésions. 
Pathogeny.  Propagation  of  the  rénal  lésion  to  the  cord  by 
means  of  the  nerves  {Troja,  Leyden).  Corroborative  experi- 
ments.  Examples  of  propagation  of  nerve-infiammations  to  the 
cord. 

Refiex  urinary  paraplegias.  Symptoms.  M.  Brown-Sé- 
quard''s  explanation.  Récent  experiments.  Inhibitory  phe- 
nomenon.  Irritation  of  the  périphérie  nerves.  Palse para- 
plegia. Descending  netiritis.  Affections  of  intestines  and 
utérus. 

Gentlemen, — I  intend  to-day  to  speak  to  you  about  tlie  varions 
affections  which  are  sometimes  designated  under  the  collective  term 
of  urinary  paraplegias.  Tbat  is  a  subject,  as  you  are  aware,  which 
has  of  late  years  given  rise  to  many  controversies.  The  debate  has 
been  a  lively  one,  conducted  at  times  with  net  a  little  beat,  on 
either  side  ;  but,  in  spite  of  ail,  it  must  be  confessed,  that  the 

'  This  lecture,  delivered  in  Juue,  1870,  was  published  in  the  'Mouvement 
Médical,'  1872. 


250  THEOEETICAL   CONSIDERATIONS. 

questions  raised  appear,  even  to-day,  to  be,  at  ail  events  in  part, 
still  wrapped  in  great  obscurity. 

In  trutli,  the  tlieoretical  aspect  of  the  subject  seems  to  hâve  been 
that  with  which  the  physicians,  who  took  part  in  the  debate, 
were  especially  preoccupied.  It  appeared  to  them  of  particular 
interest  to  investigate  by  what  means  a  pre-existing  affection 
of  the  urinary  passages  could  react  on  the  spinal  centre^  and  there 
détermine  either  an  organic  lésion  or  a  superficial  disorder  which 
should,  in  both  cases,  be  manifested  by  a  paralytic  or  paretic  state 
of  the  lower  extremities.  Much  less  attention  has,  generally 
speakiug,  been  given  to  the  establishing,  on  regular  observations, 
of  the  clinical  and  anatomo-pathological  characters  of  thèse  con- 
sécutive spinal  affections. 

No  doubt,  the  pathogenic  interprétation  is  a  point  of  the  highest 
importance  ;  it  is,  in  some  sort,  the  crowning  of  ail  pathological 
érections.  But  yet  it  is  necessary,  before  even  thinking  of  build- 
ing the  édifice,  to  carefully  examine  every  portion  of  the  ground  on 
which  it  is  to  be  founded,  and  to  be  very  certain  of  the  worth  of 
the  materials  which  shall  be  made  use  of.  Well,  gentlemen,  in  this 
particular  instance,  I  do  not  hesitate  to  déclare  that  this  ele- 
mentary  précaution  has  been  too  often  neglected  ;  and  this,  if  I 
mistake  not,  is  the  reason  that  confusion  still  reigns  over  most 
points  of  the  history  of  urinary  paraplegïa. 

You  understand,  from  the  foregoing,  that  it  is  to  a  foundation 
of  clinical  observation  and  pathological  anatomy,  I  wish,  first  of  ail, 
to  guide  you,  in  this  study  of  paraplegias  consécutive  on  diseases  of 
the  urinary  passages.  We  will  not,  however,  ncglect  the  physio- 
logical  point  of  view  ;  and  we  will  try  to  penetratc  in  this  direction 
as  far  as  may  be  possible,  in  the  actual  state  of  science. 


It  is  indispensable,  in  the  first  place,  to  prove  the  reality  of  the 
existence  of  urinary  paraplegias.  You  know  that,  underthis  name, 
we  designate  thepareôic  or  paralytic  affections  of  the  lower  exire- 
mities,  superve^iing  in  the  course  of  certain  diseases  of  the  urinary 
passages,  and  ajipearing  ta  be  connectcd  with  thèse,  as  consécutive 
ejjects,  deuter apathie  affections. 

At  the  outset  it  is  necessary,  of  course,  to  exclude  from  our  plan 
those  affections  of  the  kidneys  or  bladder  which  appear  not  as 
causes,  but,  on  the  contrary,  as  conséquences  of  a  disease  of  the 


URINAEY  PAEAPLEGIAS  :    DEFINITION.  251 

spinal  cord  ;  our  former  studies  hâve  enlightened  us  as  to  tlie 
nature  and  genesis  of  thèse  consécutive  affections  of  the  geuito- 
urinary  passages  ;  it  is  uot  necessary  to  return  to  them. 

To  accomplish  the  object  proposed,  \ve  might  quote  numerous 
observations.  The  very  number  of  the  cases  in  which  we  see  para- 
plegia  appear,  in  the  course  of  disease  of  the  urinary  passages,  is  of 
itself  enough  to  show  that  the  phenomenon  is  no  chance  coiuci- 
dence.  But  when  we  désire  to  specify  the  clinical  or  anatomical 
characters  of  thèse  paraplegias,  and  indicate  what  circuinstauces 
préside  over  their  development,  we  are  met  with  obstacles  of  ail 
kinds,  and  the  number  oîfacts  is  then  strangely  reduced.  What- 
ever  be  the  cause  of  this,  even  if  we  only  take  count  of  the  regular 
observations,  we  soon  corne  to  recognise,  on  comparing  them,  that 
they  ought  to  be  grouped  into  distinct  catégories. 

1°.  One  group  comprises  the  urinary  paraplegias  in  which  the 
spinal  cord  is  the  seat  of  an  inflammatory  lésion,  that  shows  itself, 
cluriug  life,  by  the  array  of  symptoms  which  belong  to  myelitis. 

2°.  The  second  group  includes  the  cases  in  which  the  paraplegia 
présents  itself  with  quite  différent  symptoms  ;  in  thèse  conditions 
we  remark  a  paresis,  a  weakness  of  the  lower  limbs  rather  than  a 
paralysis,  in  the  literal  acceptation  of  the  term.  The  symptoms 
are  transient,  subject  to  successive  amendments  and  exacerbations. 
The  invasion  of  phenomena  is  sometimes  rapid,  their  cessation  may 
also  take  place  suddenly.  Moreover,  beyond  the  phenomenon  of 
paresis  nothing  is  bbserved  which  would  recall  the  grave  symptoms 
peculiar  to  serions  spinal  lésions,  and  necroscopy  lias,  in  fact,  several 
times  in  such  cases  shown  the,  at  least  apparent,  integrity  of  the 
spinal  cord. 

Gentlemen,  it  is  this  form  of  urinary  paraplegia,  designated 
reflex  paraplegia,  which  was  the  especial  subject  of  the  discussion 
to  which  I  hâve  just  alluded. 

3°.  The  third  group  is  composed  of  cases  in  which  the  weak- 
ness of  the  limbs,  observed  in  the  course  of  the  urinary  disease, 
dépends,  not  on  a  spinal  affection,  but  on  a  lésion  of  the  nerves  of 
the  sacral  plexus  directly  produced,  as  it  were,  by  graduai  propa- 
gation of  the  morbid  process. 

The  two  first  groups  shall  especially  engage  our  attention  ;  as 
to  the  third  we  will  confine  ourselves  to  giving  a  rapid  account  of 
■the  cases  which  concern  it. 


252  EIEST    VAEIETY  :    MYELITIS. 

II. 

Myelilis  consécutive  on  diseases  of  the  urinary  organs. — In  sucli 
a  case  as  this,  we  hâve  to  deal  witli  a  partial  myelitis,  oocupying, 
at  least  primarily,  a  position  in  the  upper  part  of  the  lumbar 
enlargement.  This  indication  allows  you  already  to  divine  the 
array  of  symptoms  which  go  to  mark  tlie  disease. 

However,  before  proceeding  further,  it  is  necessary  to  know  the 
conditions  in  the  midst  of  which  consécutive  myehtis  hâve  their 
origin. 

Eare  amongst  women,  this  kind  of  myelitis  appears  to  be^  on  the 
other  haud,  common  enough  among  men.  Usually,  it  is  developed 
in  the  course  of  diseases  of  the  urinary  passages  of  long  duration. 

A  more  or  less  prolonged  gonorrhœa  opens  the  scène  ;  then 
follows  stricture,  which  may  occasion  the  repeated  introduction  of 
the  cathéter.  Cystitis  and  nephritis  even  may  hâve  occurred  con- 
secutively.  It  is,  as  a  rule,  in  thèse  circumstances  that  the  para- 
plegia  makes  its  appearance.  It  may  also  show  itself,  in  connec- 
tion with  disease  of  the  prostate,  with  a  cystitis  or  pyelo-nephritis 
of  calculons  origin.  We  may  regard  as  exceptional  the  case 
reported  by  H.  Leyden,  where  the  myelitis  is  said  to  hâve  followed 
upon  a  cystitis  developed  under  the  influence  of  simple  rétention  of 
urine,  occasioned  by  a  chill.^ 

Generally,  the  spinal  disorders  only  appear  several  years  after 
the  invasion  of  the  urinary  affection  ;  often  two,  five,  and  even  ten 
years  may  hâve  elapsed.  They  show  themselves  on  the  occurrence 
of  an  exacerbation  of  the  symptoms  of  the  primary  disease,  or  of  an 
unexpected  complication.  Thus,  in  a  case  reported  by  Mr.  Ogle,^ 
the  paraplegia  supervened  at  a  time  when  rénal  suppuration  had 
occurred  in  a  patient  who  had  been  afî'ected  with  calculous  cystitis 
for  five  years. 

In  an  analogous  case,  cited  by  Dr.  Gull,  the  spinal  ramollisse- 
ment showed  itself  when,  in  conséquence  of  gonorrhœa,  an  abscess 
had  formed  near  the  bulb  of  the  urethra,  and  created  a  communi- 
cation between  the  rectum  and  the  bladder.^ 

^  Leyden,  '  Ceutralblatt/  1865,  first  case. — Rétention  of  urine  for  over 
forty-eigbt  hours,  supervening  after  a  cliill.  Consécutive  cystitis.  Four 
weeks  after  tlie  rétention  the  first  symptoms  of  paralysis  of  the  lower  ex- 
tremities  appear.  On  autopsy,  red  ramolissement  of  the  lumbar  cord. 

-  'Transactions  of  the  Pathological  Society  of  London,'  1S64,  t.  xv. 

^  'Med.-Chir.  Trans.,'  t.  xxxix,  p.  200,  1856. 


MYELITIS  :    SYMPTOMS.  253 

The  case  of  H.  Leyden,  mentioned  above,  in  which  paralysis 
supervened  only  four  weeks  after  rétention  of  urine,  caused  by  a 
chill,  shows  that  the  spinal  complication  may  présent  itself  in  a 
less  dilatory  manner  in  the  course  of  disease  of  the  urinary  organs. 

I  shall  not,  of  course,  pause  to  describe  the  symptoms  of  the 
disease  of  the  urinary  organs  on  which  the  spinal  affection  follows  ; 
nor  shall  I  delay  much  over  the  phenomena  which  reveal  the 
latter  disease,  for  they  are  none  other,  as  y  ou  hâve  understood,  than 
the  symptoms  belonging  to  every  transverse  myelitis  occupying  a 
position  below  the  lumbar  swelling,  or  in  the  upper  part  of  this 
enlargement. 

Formication,  numbness,  a  feeling  of  constriction,  first  appear  in 
the  lower  limbs,  and  are  soon  followed  by  an  ansesthesia  or  an 
analgesia,  which  chiefly  affect  their  extremities  ;  the  dorso-lumbar 
pain  and  the  girdle  pain  are  rarely  absent.  A  paraplegia  with  more 
or  less  complète  flaccidness  does  not  long  delay  its  appearance;  it  is 
accompanied,  at  a  given  moment,  by  increased  reflex  excitability, 
which  i  may  give  place  to  absolute  inexcitability,  when  the  lumbar 
enlargement  is  itself  invaded  by  the  ramolHssement  ;  there  hâve  been 
cases  in  which  permanent  contracture  was  developed,  in  the  course 
of  time,  in  the  paralysed  limbs.^  The  myelitis  may  sometimes 
extend  beyond  its  primary  position,  as  is  proved  by  several  cases  in 
which  the  upper  extremities  were,  in  their  turn,  invaded  by  the 
paralysis.  Lastly,  in  grave  cases,  it  is  rather  usual  for  bedsores 
to  form  in  the  sacral  région,  and  décide  the  fatal  termination. 

The  course  of  this  form  of  myelitis  is  ordinarily  subacute. 
However,  in  the  case  reported  by  Dr.  Gull,  death  supervened  only 
fîfteen  days  after  the  invasion  of  the  paralytic  symptoms. 

The  following  is  the  information  supplied  by  autopsy.  There 
are  cases  where,  to  the  naked  eye,  the  cord  appears  to  présent  no 
altération  ;  if  the  microscope  be  then  employed,  as  was  done,  for 
instance,  in  Dr.  Gull's  case,  more  or  less  marked  histological  lésions, 
and  particularly  the  existence  of  granular  hod'ies,  are  rendered  évi- 
dent. But,  as  a  gênerai  rule,  the  most  superficial  examination 
suf&ces  to  detect,  in  the  aflfected  parts  of  the  cord,  the  diminu- 
tion of  consistency  and  change  of  colour  which  belong  to> 
ramollissement. 

It  is  not  uninteresting  to  note  that  the  lésion  in  question  appears, 

1  See,  in  particular,  the  caseof  the  celebrated  surgeon  Sauson,  reported  by 
Cruveilhier. 


254  PTîOPAGATION    OF    RENAL    LESION    TO    COED. 

at  least  at  first,  to  occupy  a  position  a  little  above  the  lumbar 
enlargemeut,  tliat  is  to  say,  in  the  place  wliere  Budge  and 
Gianuzzi  (basing  tlieir  opinion  on  anatomical  and  expérimental 
facts)  place  the  genito -spinal  centre  from  which  the  nerves  of  the 
bladder  émerge. 

It  seems  to  be  suffi ciently  proved  by  -what  précèdes  that 
transverse  myelitis  may  be  sometimes  developed  as  the  more  or  less 
direct  conséquence  of  certain  affections  of  the  urinary  passages.  It 
is  now  time  to  inquire  what  is  the  reason  of  this  relation,  or,  in 
other  terms,  by  what  mechanism  the  genito-urinary  lésion  reacts 
upon  the  spinal  centre,  and  détermines  inflammation  there. 

The  simplest  idea,  if  I  mistake  not,  which  first  of  ail  occurs  to 
the  mind  is  the  following  : — The  nerve-trunks  are  the  conduits 
through  which,  little  by  little,  the  lésion  is  propagated  in  a  cen- 
tripetal  direction,  from  the  kidneys  or  the  bladder,  to  the  spinal 
cord.  Such  is  the  opinion  adopted  by  H.  Leyden,^  in  an  interest- 
ing  study  recently  published,  and  this  opinion  strikes  me  as  very 
probable.  I  would  add  that  it  is  not  new,  for^  as  H.  Leyden, 
indeed,  acknowledges,  it  was  published,  in  1780,  by  Troja,  who, 
as  you  see,  was  not  ignorant  of  urinary  paraplegia. 

It  must,  however,  be  acknowledged  that  necroscopic  results  hâve 
not  hitherto  given  évidence  in  support  of  this  pathogenic  interpréta- 
tion. Lésions  of  the  urinary  passages  hâve  beeu  demonstrated,  on  the 
one  hand  ;  and,  on  the  other  hand,  a  spinal  lésion  ;  but  it  has  never 
hitherto  been  possible  to  find  the  trail  of  the  presumed  propagation 
of  the  inflammatory  action,  along  the  nerves. 

On  the  other  hand,  hère  is  an  expérimental  fact  which,  up  to  a 
certain  point,  may  be  appealed  to  in  favour  of  our  hypothesis.  In 
a  séries  of  studies  on  neuritis,  H.  Tiesler,  a  pupil  of  H.  Leyden," 
had  occasion  to  apply  différent  kinds  of  irritant  agents  to  the 
sciatic  nerves  of  rabbits  ;  one  of  thèse  animais  became  paraplégie 
and  succumbed  three  days  later  ;  the  autopsy  revealed  in  the  sub- 
stance of  the  sciatic  nerve,  and  in  the  place  where  the  irritation 
had  been  produced,  a  primary  purulent  focus,  and  another  focus  in 
the  veïtebral  canal  around  the  roots  of  the  nerve,  near  their  point 
of  émergence.  The  spinal  cord  in  the  same  région  was  softened  and 
infiltrated  with  granular   bodies   and  leucocytes.     The  portion   of 

'  '  Sammlung  klinisch.  Vorf.race,'  No.  2,  Leipzig,  1870. 

-  '  Ucber  Neuritis,  p.  25,  Kuuisberg,  1S69  ;  Lcjden,  loc.  cit- 


ASCENDING  NEURITIS.  255- 

the  nerve  comprised  betweeu  the  two  purulent  foci  seemed  to  be 
perfectiy  healtliy. 

This  is  the  only  experiment  fact^  witliin  my  knowledge,  wliicli 
directly  relates  to  our  subject.i  But  if  it  be  thought  well  to 
enlarge  the  field  aud  appeal  to  analogies,  we  may  iutroduce  some 
évidence  hère  the  significance  of  which  cannot  be  mistaken. 

In  inaugurating  the  lectures  of  this  year,2  we  endeavoured  to 
show  that  lésions  of  the  nervous  system  sometimes  react,  by  means 
of  the  nerves,  on  différent  parts  of  the  body  so  as  to  cause  trophic 
disorders  there.  The  converse  is  also  observed,  or,  in  other  words, 
it  may  happen  that  irritant  lésions,  primarily  occupying  the  peri- 
pheral  parts  react,  hère  again,  by  means  of  the  nerves,  on  the 
central  parts  of  the  nervous  system,  and  détermine  more  or  less 
marked  morbid  action  there.  The  facts  which  testify  to  this  effect, 
are  few  in  number  as  yet  ;  but,  they  appear  to  me  sufficiently  sig- 
nificant  to  require  a  moment's  attention. 

You  are  not  unaware  that  Graves,  in  several  passages  of  his 
writings,  expressed  the  opinion  that  inflammation  of  the  peri- 
pheral  nerves  may  be  propagated  to  the  cord.  Lallemand  mentions 
the  case  of  a  neuritis  primarily  occupying  the  brachial  plexus, 
where  the  inflammation  is  supposed  to  hâve  ascended  to  the  ence- 
phalon.  But  the  assertions  of  Graves  and  Lallemand  are  too 
vague  for  us  to  delay  longer  over  them.  Hère  are  more  explicit 
facts. ^ 

The  altérations  of  the  nerves  of  the  cmida  equina,  when  they  are 
above  the  intervertébral  ganglion,  may  ascend  to  the  cord  and  there 
détermine  the  lésions  of  grey  degeneration.  The  latter,  in  such  a 
case,  occupies  the  posterior  spinal  columus.  This  fact  has  been 
demonstrated  by  an  observation  contributed  by  M.  Cornil,* 

Eight  days  after  the  invasion  of  a  sciatic  neuritis,  developed 
under  the  influence  of  asphyxia  from  charcoal  fumes,  M,  Leudet 

1  Silice  this  lecture  was  delivered  (July,  1870)  results,  similar  to  tliose  an- 
nouiiced  by  H.  Tiesler,  hâve  been  obtaiued  by  Dr.  Feiiiberg,  "  Ueber  reflex- 
liihmungeii,"  in  'Berhner  Kiin.  Wocheiisch.,'  No.  42,  187J.  See  also  the 
important  researches  of  Dr.  Hayem  on  the  same  subject,  "Des  alicratious 
de  la  moelle  consécutives  à  l'arrachement  du  nerf  sciatique  chez  le  lapin," 
*  Arch.  de  Physiologie,'  1873.  pi.  lii,  bis,  p.  504. 

^  Référence  is  hère  made  to  the  "Leçons  sur  les  troubles  trophiques  con- 
sécutifs aux  lésions  du  Système  nerveux,"  delivered  in  1870. 

^  See  Leudet,  'Archives  Générales  de  Médicine/  1865,  t.  ii,  p.  52S. 

*  Sec  Bouchard,  '  Des  Dé^'éuérations  secondaires,'  p.  42. 


S56  ASCENDING    NEURITIS. 

remarked  paretic  weakening  to  supervene  at  first,  in  the  lower 
extremity  corresponding  to  tlie  seat  of  the  neuritis,  then  extending 
to  the  limb  of  the  opposite  side,  and,  lastly,  to  the  upper  extre- 
mities.i 

H.  Leyden  has  quoted  from  H.  Benedikt  the  following  fact  : — 
A  fracture  of  the  neck  of  the  humérus,  ending  in  pseudarthrosis, 
had  been  the  source  of  acute  permanent  pains  occupying  the  whole 
length  of  the  lower  extremity  of  the  side  corresponding  to  the 
fracture.  Shortly  after,  complète  paraljsis  with  muscular  atropliy 
supervened  in  the  upper  extremity  of  the  same  side.^ 

In  a  very  interesting  case,  related  by  M.  Duménil — who,  I 
should  warn  you,  has  given  a  différent  interprétation  of  it  from  that 
which  I  propose — there  were  successively  observed  in  one  of  the 
extremities,  first,  a  sciatic  neuritis,  next  a  paraplegia,  and  lastly  a 
paralysis  of  the  upper  limbs.  Muscular  atrophy  with  decrease  of 
electric  contractility  attacked  the  motor-paralysed  limbs,  in  succes- 
sion. The  tongue,  itself,  was  finally  involved.  The  autopsy 
revealed  some  remarkable  lésions  principally  occupying  the  central 
grey  substance,  the  white  substance,  on  the  contrary,  being  scarcely 
affected.^ 

I  shall  lastly  mention  a  case  of  my  own  which  I  contributed  to 
Brown-Séquard's  'Journal  de  Physiologie,'  for  1856.  It  refers  to  a 
nerve  lésion  of  the  forearm,  which  determined,  firstly,  a  neuritis  of 
the  peripheral  end,  followed  by  atrophy  of  the  muscles  of  tlie  hand 
and  by  a  pemphigoid  éruption.  Afterwards,  the  upper  extremity 
of  the  opposite  side  was,  in  its  turn,  stricken  with  atrophy  and 
ansesthesia.^ 

^  Leudet,  loc.  cit.  "  Leyden,  loc.  cit.,  p.  21. 

3  'Gazette  hebdomadaire,'  1866. 

■*  The  following  case  ought,  I  believe,  to  be  considered  as  an  example  of 
ascending  neuritis,  followed  by  transverse  myelitis.  M.  X — ,  aged  fift.j,  had 
amputation  of  the  left  thigh  performed  at  the  âge  of  twenty.  During  several 
monlhs  he  feit,  in  the  stump,  acute  pains,  formication,  and  sometimes  startings, 
when,  one  day  in  August,  1875,  vesical  paralysis  and  lumbar  pains  supervened. 
Shortly  after  formication  and  startings  were  experieneed  in  the  right  lower 
•extremity  which,  together  with  the  stump,  soon  became  stricken  with  motor 
paralysis  and  llaccidness.  After  some  days  of  treatment,  the  patient  recovered 
the  functions  of  the  bladder,  and  some  weeks  after  he  was  able  to  leave  his 
bed  and  to  walk,  of  course  with  the  help  of  crutches.  A  year  after,  he  walked 
still  better  ;  but  in  the  right  lower  extremity  a  certain  degree  of  permanent 
rigidity  had  been  produced.  On  forcibly  flexing  the  point  of  the  foot,  very 
markcd  trépidation  was  without  fail  causcd  in  this  limb. 


REPLEX   UKINART  PARAPLEGIAS.  257 

It  is  at  least  very  probable  that,  in  ail  thèse  cases,  tlie  nerves 
hâve  been  the  seat  of  inflammatory  action  whicli  was  propagated 
to  the  cord  and,  according  to  ail  probability,  to  the  central  grey 
substance.  In  fact,  in  most  of  thèse  cases,  the  ansesthesia  and 
muscular  atrophy  of  the  paralysed  liinbs  seem  to  indicate  that 
we  had  hère  a  form  of  subacute  central  myelitis,  to  which  I 
intend  soon  to  call  your  attention,  and  in  which  the  muscular 
atrophy  and  différent  disorders  of  sensibility  appear  to  be  constant 
phénoménal 

III. 

The  cases  which  constitute  the  second  group  of  urinary  para- 
plegias  are  tliose  which  Rayer,  Brown-Séquard,  and  M.  R.  Leroy 
d^Etiolles  hâve  chiefly  had  in  view  in  their  descriptions.  Paralysis 
shows  itself  hère,  uuder  precisely  the  same  circumstances  as  in  the 
preceding  cases,  and,  in  its  etiology,  we  again  find  diseases  of  the 
urethra,  of  the  bladder,  prostate,  and  kidneys. 

Nothing,  therefore,  in  this  respect,  séparâtes  thèse  from  those. 
This,  however,  does  not  hold  good  as  regards  their  clinical  charac- 
ters.  They  differ  radically,  as  I  hâve  indicated,  from  those  which 
distinguish  urinary  myelitis. 

The  picture,  drawn  by  Brown-Séquard,  of  what  he  calls  rejlex 
jjarapleffia,  has  excited  sufîicient  attention  to  render  any  long  discus- 
sion of  this  subject  unnecessary.  I  shall  confine  myself  to  remind- 
ing  you  of  the  following  characters  ;  they  will  enable  you  to  group 
the  différences  which  exist  between  this  form  of  urinary  paraplegia 
which,  in  reality,  is  rather  frequently  observed  in  ordinary  practice, 
and  that  which  is  connected  with  partial  myelitis  arising  from  a 
rénal  or  vesical  cause. 

The  paralysis  uever  extends  to  the  upper  extremities  ;  besides,  we 
hâve  hère  to  deal  with  a  paretic  weakness  of  the  limbs  rather  than 
with  paralysis  properly  so  called  ;  the  reflex  power  of  the  cord  is 
never  augmented  ;  never  is  paralysis  of  bladder  or  rectum  superadded 
to  that  of  the  lower  extremities  ;  neither  muscular  spasms  nor 
contracture  are  observed  ;  dysaesthesia,  like  ansesthesia,  is  altogether 
absent  ;  no  bedsores  nor  any  kind  of  trophic  disorders  are  produced, 
and  the  absence  of  dorsal  pains,  and  of  ail  sensations  of  abdominal 
constriction  is  expressly  remarked.  Lastly,  and  this  is  a  character 
well  worth  noticing,  there  is  often  a  rapid  and  sometimes  even  a 
'  See  Lectures  IX — XIV  on  "  Spinal  Amyotrophies." 

VOL.   II.  17 


258       PATHOGENT  OF  EEFLEX  PAEAPLEaiAS. 

complète  modification  of  the  paralytic  phenomena,  under  the  in- 
fluence of  an  amendment  in  the  affection  of  the  urinary  passages. 

In  short,  gentlemen,  as  you  see,  the  symptoms  are  relatively  little 
marked,  when  compared  with  those  depending  on  urinary  myelitis. 
The  afi'ection  in  question  never,  in  fact,  endangers  life  of  itself  ;  it 
does  not  appear  to  hâve  ever  resulted  in  myelitis,  and  in  the  (not  very 
explicit)  cases  where  autopsy  has  been  performed,  the  cord  has 
always  appeared  free  from  altération.  It  is  true  that,  up  to  the 
présent,  vérification  by  microscopic  study  has  been  déficient.  But, 
on  the  other  hand,  the  rapidity  of  the  improvements,  and  even  of 
the  recoveries,  observed  in  a  number  of  cases  is  such  that  it  is  very 
improbable  that  microscopical  investigation  could  hâve  added  any- 
thing  to  the  examination  made  with  the  naked  eye. 

Nevertheless,  gentlemen,  this  paralytic  debility  of  the  lower 
extremities,  developed  in  conséquence  of  a  disease  of  the  urinary 
passages,  is,  at  ail  events,  a  very  curious  phenomenon,  and  it  is  not 
surprising  that  it  has  so  keenly  engaged  the  attention  of  physiolo- 
gists.  You  are  not  ignorant  of  the  interprétation  proposed  by  M. 
Brown-Séquard.  It  is  based  upon  an  experiment.  Ligature  of  the 
hilum  of  the  kidney  has,  it  would  appear,  the  effect  of  determining 
a  sort  of  prolonged  contracture  of  the  vessels  of  the  spinal  cord  and 
of  its  envelopes,  and  it  is  in  conséquence  of  the  spinal  ansemia  thus 
developed  in  a  reflex  manner  that  paraplegia  is  produced.  The 
validity  of  the  experiment  has  been  contested  by  Dr.  GuU.  I  will 
not  insist  upon  this  point,  not  having  any  personal  experiment  to 
mention;  but  I  cannot  omit  giving  you  a  brief  description 
of  the  results  of  the  most  récent  experiments,  which,  if  they  do  not 
completely  reveal  the  mechanism  of  reflex  paraplegias,  at  ail  events, 
if  I  mistake  not,  demonstrate  their  reality. 

The  phenomena  of  inJiibition,  observed  in  conséquence  of  the 
irritation  of  certain  nerves,  are  well  known  to  you  ;  you  know  how 
irritation  of  the  vagus  stops  the  heart,  that  of  the  splanchnic  nerve 
paralyses  the  intestine,  and,  lastly,  hovr  that  of  the  superior  laryn- 
gcal  nerve  causes  momentary  suspension  of  the  respiration.  But, 
it  is  probably  less  generally  known  that  irritation  of  certain  points 
of  the  cord  or  of  the  spinal  nerves  is,  also,  capable  under  certain 
circumstances  of  forming  a  hindrance  to  the  regular  functions  of 
the  cord,  of  momentarily  abolishing,  for  instance,  the  play  of  the 
motor  functions  and  reflex  acts. 

The  encephalon  of  a  frog  having  been  destroyed,  if  you  irritate 


INHIBITOEY    PHENOMENA.  259"^ 

the  spinal  cord,  in  its  lower  région,  by  a  caustic  application,  the 
reflex  acts  are  diminished,  to  a  remarkable  extent,  in  the  upper 
extremities  during  the  whole  time  that  the  irritation  lasts.  This 
experimentis  due  to  M.  Herzen.  Hère  is  another  which  we  owe  to 
the  same  physiologist  :  the  brain  and  cord  of  a  frog  liaving  been  de- 
stroyed  to  a  level  with  the  brachial  plexus,  if  you  then  irritate.  in 
any  manner,  the  sciatic  nerves  of  the  left  side,  for  instance,  as  long 
as  the  irritation  persists,  the  reflex  movements  are  abolished  in  the 
lower  extremity  of  the  right  side.  If  the  electric  stimulus  be  em- 
employed,  amongst  other  tests,  the  movements  reappear  immediately 
on  the  current  ceasing.^ 

The  experiments  of  H.  Lewisson"  probably  possess,  from  my 
point  of  view,  still  greater  interest  than  the  foregoing.  His  first 
séries  is,  indeed,  little  more  than  a  reproduction  of  the  experiments 
of  M.  Herzen,  with  variations  and  some  modifications,  which  render 
the  results  more  délicate  and  more  précise. 

We  shall  only  refer  to  the  facts  which  touch  most  nearly  the 
subject  that  occupies  us.  One  of  the  first  points,  demonstrated  by 
this  expérimenter,  is  the  non-existence  of  reflex  paraplegia,  consécu- 
tive on  the  extirpation  of  the  kidney.  The  experiment  of  Com- 
liaire,  which  lias  so  long  served  as  a  stock-in-trade,  does  not,  it  is 
asserted,  give  the  results  which  liave  been  attributed  to  it.  But  if, 
after  having  laid  bare  the  utérus,  the  kidneys,  the  empty  bladder  of 
a  rabbit,  you  press  thèse  différent  organs  more  or  less  strongly 
between  the  fingers,  motor  paralysis  is  produced  in  the  lower  ex- 
tremities, which  persists  as  long  as  the  pressure  is  kept  up,  and 
even  for  some  time  after.  The  constriction  of  a  loop  of  the  intestines 
détermines,  also,  the  same  effects,  and  is  likewise  followed  by  tem- 
porary  paraplegia. 

I  am  far  from  denying  that,  in  many  respects,  the  application 
of  thèse  results  to  the  explanation  of  the  paraplegias  termed 
reflex  is  open  to  criticism.  It  may  be,  for  instance,  objected 
that  the  paralysing  influence  caused  by  pressure  of  the  viscera 
ought,  necessarily,  to  exhaust  itself  at  the  end  of  a  certain  time  ; 

*  Herzen  :  '  Expér.  sur  les  centres  modérateurs  de  l'action  réflexe,'  Turin, 
1864. 

2  Lewisson,  "Ueber  Hemmung  der  Thatigheit  der  motorischen  Nerven- 
centren  durch  Reizung  sensibler  Nerven."  Du  Bois,  '  Archiv,'  1869,  s.  255, 
266.  Nothuagel,  'Vircliow's  Arcbiv,'  Jan.,  1870.  '  Centraiblatt,'  1869, 
p.  623. 


260  UEINAKY    PAKAPLEGIAS  :    THIED   GEOUP. 

that  the  irritation  of  the  peripheral  nerves,  resulting  from  cystitis  or 
nephritis,  is  not  exactly  comparable  to  compression  of  the  bladder 
or  kidney.  Ail  this  is  perfectly  true,  but,  without  going  so  far  as 
to  admit  that  there  is,  between  thèse  two  classes  of  facts,  a  com- 
plète identity,  it  is  allowable  to  recognise  the  analogies  whicli  bring 
them  together,  and  to  hope  that  expérimental  facts  will  some  day 
guide  us  to  the  interprétation  of  the  clinical  phenomena. 

IV. 

As  I  announced,  at  the  beginning,  there  exists  a  third  group  of 
urinary  paralyses.  The  cases,  few  in  number,  which  compose  it  are, 
so  far,  examples  of  false  paraplegia,  at  least  in  this  respect  that  it 
is  not  the  cord  which  is  attacked. 

In  such  cases,  the  phenomena  take  place  as  in  the  well-known 
example  reported  by  H.  Kussmaul.^  In  that  instance,  descending 
neuritis,  directly  propagated,  had  become  developed  owing  to  a 
serions  inflammation  of  the  urinary  passages,  and  had  occupied  the 
lumbar  and  sacral  plexus.  During  life,  besides  paresis  of  the  lower 
extremities,  acute  pain  had  been  remarked  along  the  course  of  both 
sciatic  nerves.  Cases  of  this  kind  rarely  make  their  appearance,  I 
repeat,  contrary  to  the  opinion  of  Remak,  who  seems  to  believe  that 
this  is  the  usual  form  of  urinary  paraplegias. 

The  pathogenic  modes  which  hâve  just  been  reviewed  are  not  the 
cnly  examples  which  might  be  appealed  to  in  order  to  explain  the 
development  of  paraplegias  consécutive  on  diseases  of  the  genito- 
urinary  organs.  Thus,  in  cases  reported  by  Dr.  Gull,  a  phlebitis 
consécutive  on  an  abscess  of  the  lesser  basin,  itself  being  caused 
by  an  ulcérons  cystitis,  was  propagated  to  the  intra-spinal  veins  ; 
there  resulted,  by  a  mechanism  easily  understood,  a  partial  myelitis, 
which  was  soon  followed  by  death. 

You  are  aware,  gentlemen,  that  uro-genital  lésions  hâve  not 
alone  the  power  of  determining  paralyses  of  the  kind  which  we  hâve 
just  been  studying.  Varions  affections  of  the  intestine  or  of  the 
utérus  may  also,  though  more  rarely,  be  followed  by  the  same  con- 
séquences. I  confine  myself,  for  the  moment,  to  pointing  ont  this 
fact  to  you  :  it  deserves  to  engage  your  attention,  in  a  spécial 
manner. 

1  '  Wurzburg.  Verhand.,'  iS68. 


LECTURES  XVII  AND  XVIII. 

MÉNIÈRE'S  VERTIGO  =  VERTIGO  AB  AURE  L^SA. 

SuMMARY. — A  case  of  Ménière's  vertigo.  Description.  Habituai 
vertigo  increased  ly  motion.  Us  characters  :  paroxysmal 
exacefhations  ;  subjective  translation  movements.  OUI  lésions 
of  tle  ears  ;  outfloio  of  pus,  altération  of  tympan.  WalUng 
and  standing  impossible,  évolution  of  tJie  disease.  Complica- 
tion :  hystericalfits. 

Historical  sketch.  Vertigo  of  Menière  as  yet  Utile  Jcnoivn. 
Biagnosis  ;  apoplectif or  m  cérébral  congestion  ;  epileptic  petit 
mal;  gastric  vertigo.  Relation  between  the  sudden  development  of 
noises  in  the  ears  and  the  invasion  of  sensations  of  giddiness. 

Diseases  of  the  car:  labyrinihic  otitis ;  médian  otitis, 
catarrh,  8fc.     Prognosis.     Cure  by  deafness.     Treatment. 

Repîitedly  incurable  diseases.  Examples  of  cure.  Case  of 
Ménière' s  vertigo.  Situation  of  the  patient  in  May,  1875; 
permanent  sensations  of  giddiness  ;  crises  annoîonced  by  shrill 
whistling.  Motor  hallucination.  Treatment  by  sulphate  of 
quinine:  doses,  effects,  remarkable  improvement.  Another 
example  of  amélioration  due  to  the  prolong ed  use  of  sulphate  of 
quinine. 

I. 
Gentlemen, — I  wish  to  call  your  attention  to  a  case  which,  in 
my  opinion,  is  very  interesting,  aud  to  which  you  hâve  probably 
not  often  seen  a  parallel  in  the  hospitals.  The  symptoms  appear 
hère  in  a  very  marked  manner,  but  abnormally  in  some  respects,  so 
that  the  affection  in  question  is  rendered  to  a  certain  extent  difficult 
of  diagnosis.  You  can  at  once  observe,  de  visu,  the  utterly  scared 
look  which  the  physiognomy  of  the  patient  présents.  If  you 
approach  her  bed,  she  at  once  gives  signs  of  great  anxiety  ;  you  see 
her,  at  the  slightest  shake,  seize  and  cHng  to  the  objects  around, 
as  though  she  felt  in  danger  of  falhng. 


262  CASE  OF  ménière's  vertigo. 

The  fact  is  that  she  is  under  the  influence  of  a  state  of  giddiness 
■which  is,  as  it  were,  perpétuai,  and  which  the  slightest  motion 
exaspérâtes.  This  vertigo  she,  herself,  describes  in  picturesque 
terms.  It  is,  she  says,  the  sensation  which  one  might  feel  when 
standing  on  the  summit  of  a  high  tower,  unprotected  by  any  rail  ; 
or  such  as  might  be  caused  by  the  sight  of  a  précipice.  This 
vertigo,  I  repeat,  is  almost  incessant;  it  exists  by  night  as  well  as 
by  day,  when  in  dorsal  decubitus  as  well  as  when  standing  upright. 
The  latter  position,  however,  augments  it  enormously.  This  also 
happens,  I  hâve  said,  from  the  least  shake  of  the  bed  and,  in  a 
moment,  when  the  patient  shall  be  lifted  in  order  to  be  taken  to  the 
ward  which  she  usually  occupies,  you  willperhapshearhershrieking 
wildly,  owing  to  an  exaspération  of  the  sensations  of  giddiness. 

At  times,  in  the  midst  of  apparent  calm,  and  without  any 
provocation  whatever,  the  patient,  as  you  may  doubtless  soon 
remark,  suddenly  gives  a  start.  If  she  be  then  asked  what  is  the 
reason  of  this  abrupt  movement  she  invariably  replies  that  she  has 
just  had  her  fit.  The  fact  is,  that  besides  the  state  of  habituai 
giddiness  which  I  endeavoured  to  depict  a  moment  ago,  she  is 
subject  to  paroxysmal  exacerbations  of  the  vertigo  which  constitute 
a  kind  of  fits.  Thèse  appear  to  be,  especially,  characterised  by 
the  sensation  of  a  sudden  translation  movement,  not  of  the  sur- 
rounding  objects,  but  of  the  patient  herself — an  altogether  sub- 
jective sensation,  the  only  exterior  sign  of  which  is  the  starting. 
Still,  consciousness  is  not  at  ail  lost,  and  the  patient,  on  emerging 
from  her  fit,  «an  give  an  account  of  ail  she  experienced.  Sometimes, 
and  most  frequently,  it  seems  to  her  that  she  makes  a  summersault 
forward  ;  at  other  times  the  summersault  is  made  backward.  Lastly, 
and  most  rarely,  she  expériences  a  sensation  as  if  her  body  were 
rapidly  spinning  around  on  its  vertical  axis — the  rotation  always 
taking  place  from  left  to  right.  Whichever  may  occur,  this  species 
of  motor  hallucination  is  always  followed  by  keen  anxiety,  facial 
pallor,  and  cold  sweats.  Finally,  nausea  and  also  sometimes 
vomitings  terminate  the  crisis  ;  after  which  the  state  of  giddiness 
re-descends,  so  to  speak,  to  its  normal  standard. 

In  addition  to  the  phenomena  which  hâve  just  been  mentioned 
there  is  yet  another,  gentlemen,  which  deserves  to  be  noticed  in  a 
spécial  way,  because,  in  relation  to  this  subject,  it  is  I  think,  of 
capital  importance,  especially  from  a  diagnostic  point  of  view.  I 
allude  to  a  whistling  sound  which  occupies  both  ears  of  our  patient, 


DESCRIPTION.  263 

but  is  prédominant  in  the  left.  This  whistUng  exists  almost  con- 
stantly  to  a  certain  degree,  but  becomes  exasperated  at  times  and 
tlien  occasionally  acquires  an  extrême  shrillness.  In  fact,  the 
patient  sometimes  confounds  it  with  the  shriek  of  the  steam-whistle 
sounded  in  the  railway  station  adjacent  to  the  hospital;  she  has  had 
to  inquire  of  her  neighbours  to  make  certain  of  the  fact.  This 
exacerbation  of  the  habituai  whistling,  gentlemen  (and  this  point 
deserves  to  be  remarked),  always  heralds  the  near  arrivai  of  the  fit 
of  giddiness.  When  it  acquires  this  shrill  character,  the  "  sum- 
mersaults  '^  are  imminent. 

The  constant  appearance  of  the  symptoms^under  the  circumstances 
just  indicated,  naturally  directed  our  attention  to  the  ear,  and  this  is 
what  we  found  on  examination  :  i°.  From  time  to  time,  for  a  long 
period,  there  cornes  an  outflow  of  pus,  mixed  with  blood,  from  both 
ears,  but  especially  from  the  left  ;  2°,  on  the  right,  the  membrana 
tympani  is  thickened,  covered  with  greenish  deposits  ;  on  the  left,  it 
has  disappeared,  and  is  replaced  by  fungoid  growths.  On  this 
side,  there  is  considérable  loss  of  hearing  power.  Thèse  several 
lésions  hâve  been  duly  verified  by  a  physician  more  particularly 
versed  in  the  study  of  diseases  of  the  ear,  who  has  obligingly  given 
us  bis  assistance. 

Under  the  extraordinarily  marked  form  which  it  assumes  in  the 
case  of  our  patient,  this  symptomatic  group,  which  is  commonly 
inown  by  the  name  of  Mêniere's  disease,  is  undoubtedly  difficult  of 
récognition  ;  but  this  is  not  the  case  if,  by  a  study  of  its  antécé- 
dents, you  refer  back  for  some  years,  namely,  to  an  epoch  when  the 
crises,  dissociated,  as  it  were,  made  their  appearance  with  characters 
which  recall  the  classic  description  in  its  principal  features. 

It  is  not  much  more  than  six  years  ago,  in  fact,  since  the  state 
of  giddiness  was  established  in  a  permanent  manner,  hère,  so  as  to 
render  it  impossible  for  the  patient  to  walk  or  even  to  stand,  and 
necessitating  confinement  to  bed.  Before  this  epoch,  the  fits  were 
long  distinct,  separated  by  more  or  less  lengthy  intervais,  during 
which  everything  seemed  to  fall  into  good  order.  This  is  what  we 
learn  from  the  détails  of  the  observation  which  M.  Debove  has 
noted  with  the  greatest  care. 

The  first  symptoms  must  be  dated  from  about  the  âge  of  seven- 
teen,  and  G —  is  now  fifty-one.  At  first,  the  left  ear  suffered,  the 
affection  beiug  chiefly  marked  by  shooting  pains  which,  oftentimes 
disturbed  her  sleep  ;  the  runniugs  of  pus  mixed  with  blood  hâve 


264  CASE  op  ménière's  vertigo. 

been  fréquent  from  this  epoch.  Por  a  long  time  the  patient  was 
placed  under  the  care  of  Ménière.  The  fits  of  giddiness,  at  first 
rare  and  of  little  intensity,  hâve  become  gradually  more  marked 
and  doser  ;  but,  from  the  very  beginning,  they  appear  to  hâve  pre- 
sented,  though  in  an  abridged  form  it  is  true,  ail  the  more  marked 
characteristics  which  distinguish  them  to-day.  Thus,  the  patient 
remembers  quite  well  that,  between  the  âges  of  twenty-five  and 
thirty-eight,  she  often  experienced,  whilst  sitting,  very  intense 
buzzing  in  the  ear^  and  immediately  the  chair  seemed  to  break 
down  under  her.  She  gave  a  cry,  rose  up  quickly,  and  ail  was 
over.  Afterwards,  about  the  âge  of  thirty-eight,  the  premonitory 
buzzing  gave  place  to  shrill  whistling,  and,  at  the  same  time,  nausea 
and  vomiting  began  to  form  an  intégral  part  of  the  fits.  The 
latter  often  supervened  in  the  street  ;  Gir —  then  habitually  experi- 
enced  the  sensation  oîfallingforward,  and,  in  order  not  to  fall,  was 
obliged  to  lean  against  the  wall. 

They  frequently  came  on  at  home,  during  work  hours,  and  Gir — 
in  the  course  of  the  years  preceding  her  admission  into  this 
hospital,  had  adopted  the  singular  custom  of  placing  herself, 
during  the  time  she  spent  at  home,  in  such  a  position  as  that  her 
head  was  slightly  thrown  back,  whilst  her  legs  were  somewhat 
raised.  Thanks  to  this  position,  which  is  still  habituai  to  her,  the 
vertigo,  she  déclares,  was  less  fréquent  and  less  distressing. 

Towards  the  âge  of  forty-five,  the  fits  had  approximated  to  such 
an  extent  as  to  become  subintrant,  so  to  speak  ;  shortly  afterwards, 
the  patient  was  admitted  to  the  Salpêtrière,  where  she  has  remained 
for  six  years,  under  our  eyes,  in  the  lamentable  state  in  which  you 
see  her  to-day. 

In  order  not  to  overload  the  clinical  picture,  which  is  already  a 
complicated  one,  I  hâve  purposely  neglected  to  mention  certain 
nervous  accidents  which  G —  has  experienced  during  a  considérable 
portion  of  her  life,  and  traces  of  which  she  bears  to-day  ;  I  refer  to 
fits  of  convulsive  hysteria  which  hâve  often  intermingled  with  fits 
of  vertigo  ah  aure  lœsd,  without,  however,  ever  becoming  con- 
founded  with  them.  At  présent,  the  convulsive  fits  hâve  dis- 
appeared,  and  the  hysteria,  for  many  years,  has  only  been  repre- 
sented  by  an  incomplète  hemiansesthesia  and  ovaralgia,  of  the  left 
side. 


HISTORICAL    CONSIDERATIONS.  265* 

II. 

Very  important  works  hâve  been  published  concerning  the 
symptomatology  of  the  vertigo  ah  mire  lasd.  As  examples,  I  will' 
mention  the  communication  made  to  the  Académie  de  Médecine, 
June  8,  1861,  by  Ménière,  who,  as  you  are  aware,  was  undoubtedly 
the  iirst  in  the  field  :  then  the  description,  traced  with  a  master- 
hand,  by  Trousseau  in  différent  places  in  his  '  Clinique  Médicale  de 
FHôtel  Dieu  (t.  ii,  p.  28,  t.  iii,  p.  11).  I  would  also  cite  a  very 
important  memoir  by  Dr.  Knapp  (of  New  York),  where  most  of  the 
éléments  noted  in  référence  to  this  subject,  up  to  the  présent,  hâve 
been  collected  (Knapp  and  Moos,  '  Archives  of  Ophthalmology  and 
Otology,'  t.  i.  No.  I,  New  York,  1870);  lastly,  an  excellent  article 
published  by  M.  Duplay  in  the  '  Archives  de  Médecine.' 

Nevertheless,  I  believe  I  may  assert  that,  in  spite  of  thèse  works, 
a  knowledge  of  the  pathological  condition  in  question  has  not  yet 
entered,  as  it  ought,  into  every-day  practice.  Although  cases  of. 
Ménière's  disease  are  not  rare,  far  otherwise,  at  least  in  civil  prac- 
tice, they  are  nearly  always  misconstrued,  connected  as  they  are 
with  more  common  disorders,  as,  amongst  others,  with  apoplecti- 
form  cérébral  congestion,  or  apoplectic  stroke,  epileptic^e^i^  mal^ 
or,  again,  and  chiefly  with  gastric  vertigo.  I  hâve,  for  my  own  part, 
often  witnessed  mistakes  of  this  kind  ;  as  an  example,  I  will  men- 
tion the  case  of  a  patient,  whom  I  hâve  attended,  and  who,  having- 
fallen  on  the  Place  de  la  Bourse,  owing  to  a  fit  of  labyrinthic  ver- 
tigo, had  been  treated  by  bloodletting.  The  real  character  of  the 
disease  was  not  recognised  until  very  late,  at  a  time  when  the  fits,. 
which  were  of  great  intensity,  had  been  already  very  frequently  re- 
produced.  Complète,  absolute  deafness  of  both  ears  put  an  end  ta 
ail  the  symptoms.  I  might  also  cite  the  case  of  a  young  American  lady 
who  had,  for  many  years,  been  considered  as  epileptic,  and  conse- 
quently  treated,  without  indeed  the  least  improvement,  by  large  dose& 
ofbromideof  potassium.  Itwould  be  easy  for  me  tomultiply  examples. 

The  error  in  some  cases  is,  to  a  certain  point,  justified  by  the 
difficulties,  often  very  serious  ou  es,  which  may  stand  in  the  way  of 
the  diagnosis.  I  think,  however,  that,  as  a  rule,  the  labyrinthic 
vertigo  présents  itself  with  a  sufficiently  characteristic  aspect  tO' 
allow  of  its  identity  being  determined  without  over-much  difficulty. 
I  request  your  permission  to  note  the  principal  characters,  for  \i 
I  should  succeed  in  graving  them  on  your  mind,  they  would  enablo 


266  OHAEACTERS  OF    LABYRINTHIO  VEETIGO. 

you,  I  am  convinced,  nearly  always  to  steer  clear  of  the  rocks  which 
I  hâve  just  described. 

In  the  first  ijlace,  I  would  point  out  the  close  relation  which  exists 
between  the  sudden  development  of  noises  in  the  ear,  or  the  abrupt 
exaspération  of  thèse  noises,  and  the  invasion  of  the  sensations  of 
giddiness.  In  reality,  one  of  the  spécifie  features  of  Ménière's  dis- 
ease  is  that  it  is,  of  necessity,  heralded  and  accompanied  by  the 
noises  in  question.  Undoubtedly,  tinkling,  buzzing,  and  whistling 
noises  in  the  ear  constitute  a  somewhat  commonplace  phenomenon, 
which  frequently  accompanies  différent  kinds  of  vertigo  other  than 
that  connected  with  Ménière's  disease,  but  in  the  latter  affection  it 
acquires,  at  the  moment  of  the  fit,  an  intensity  and  prominence 
which  are  certainly  not  elsewhere  observable.  According  to  the 
statements  of  the  patients  it  is  "  the  shrill  sound  of  a  locomotive 
whistle,"  or  a  rattle,  like  ''  the  violent  shaking  of  a  sack  of  nails/' 
or,  again,  "  the  crackle  of  fireworks,  or  a  fusillade."  This  noise 
occupies  one  of  the  ears,  either  exclusively  or  especially.  It  ceases 
with  the  fit  of  giddiness  in  récent  or  shght  cases  ;  but  sooner  or 
later,  if  the  case  is  grave,  it  becomes  persistent,  in  the  intervais,  in 
the  form  of  a  buzziug  or  a  tinkling,  more  or  less  disagreeable  ;  the 
affected  ear,  besides,  does  not  fail  to  soon  présent  a  more  or  less 
marked  and  permanent  deafness. 

When  the  attention  of  the  physician  has  been  once  aroused  by 
thèse  phenomena,  an  examination  of  the  auditory  apparatus  always 
enables  him  to  recognise  the  existence  of  local  symptoms,  denoting 
either  idiopathic  labyrinthic  otitis,  or  sclerematous  médian  otitis, 
with  anchylosis  of  the  ossicula,  propagated  to  the  vestibule  and 
labyrinth,  or  again,  a  simple  catarrh  of  the  ear,  as  shown  in  an 
observation  pubhshed  by  Mr.  Green  ('Boston  Med.  and  Surg. 
Journal,'  aist  Jan.,  1869)  and  quoted  by  Mr.  Knapp.  There  is 
reason  besides  to  believe,  from  the  gênerai  aspect  of  the  facts, 
that  any  pressure  exercised  upon  the  tympanum  and  carried  on  to 
the  labyrinth  by  the  chain  of  ossicula,  suffices  to  détermine  the 
symptoms  of  Ménière's  vertigo. 

With  respect  to  the  vertigo,  considered  in  itself,  it  also  offers 
some  spécial  characters.  Most  frequently,  if  I  am  to  judge  from 
ten  or  a  dozen  of  my  own  cases,  it  is  the  sensation  of  a  translation 
movement  of  the  whole  body,  from  behind  forward,  or  from  before 
backward,  so  as  to  simulate,  according  to  the  case,  a  fall  forward  or 
backward  ;  or  again,  when  a  feeling  of  rotation  round  a  transverse 


MOTOE   HALLUCINATIONS.      NAUSEA.  267 

axis  is  added,  it  is  a  genuine  suminersault,  as  if  from  a  spring-board. 
Occasionally,  on  the  contrary^  the  rotation  of  the  body  seems  to  take 
place  round  a  vertical  axis,  sometimes  from  left  to  right,  sometimes 
from  right  to  left.  There  are  patients  who,  in  their  différent  fits, 
imagine  that  they  expérience  sometimes  one,  sometimes  another  of 
thèse  différent  modes  of  rotation.  Ail  this,  remark  it  well,  refers, 
generally,  to  purely  subjective  movements,  genuine  hallucinations, 
which  are  only  betrayed  externally  by  a  start,  a  movement  of 
surprise,  occasionally  by  the  patient's  catching  at  adjacent  objects 
or  sittiûg  down,  for  fear  of  falling.  But  it  may  happen  that  a  fall 
actually  takes  place,  and  that  the  patient  may  be  thrown  violently 
down,  in  a  direction  corresponding  with  the  vertigo  sensation.  In 
connection  with  this,  I  may  mention  the  case  of  a  lady  who,  in  her 
fits,  always  felt  herself  precipitated  head  foremost  and  who,  in  fact, 
in  one  of  them,  did  fall  heavily  on  her  face,  breaking  the  bones  of 
her  nose.  I  am  aware  that  the  feeling  of  rotation  or  translation 
may  be  observed  in  the  most  différent  kinds  of  vertigo,  but  I 
believe  I  can  affirm  that  you  never  fiad  it  there  either  so  marked, 
or  so  constant,  as  it  is  in  Méuière's  vertigo. 

It  is  important  to  remark  that,  during  the  crisis,  whatever  may 
be  its  intensity,  the  patient  absolutely  préserves  perfect  conscious- 
ness  of  his  actions,  and  that,  once  the  first  effects  of  the  seizure 
hâve  been  dissipated,  he  is  immediately  in  a  position  to  render, 
without  hésitation,  an  account  of  ail  that  he  has  felt. 

As  accessory  phenomena,  I  would  point  ont  the  following  : — 
Nausea  and  vomiting,  almost  constantly,  mark  the  end  of  the 
crisis  ;  whilst  it  lasts,  the  face  is  pale,  the  skin  cold  and  covered  with 
sweat  in  such  a  way  as  to  reproduce  the  semblance  of  syncope  rather 
than  that  of  an  apoplectic  stroke.  A  more  or  less  acute  cephalalgia 
may  exist  for  the  moment.  There  is  never  any  difïiculty  of  utter- 
ance,  nor  muscular  spasms  in  face  or  limbs  ;  never  in  the  extremities 
any  formication  nor  numbness,  nor  sensations  like  an  aura,  never  any 
temporary  pareses  nor  paralyses. 

At  the  outset,  that  is  to  say  when  Ménière's  disease  is  yet  but 
beginning,  the  vertigo  appears  under  the  form  of  distinct  crises, 
of  short  duration,  separated  by  intervais  of  absolute  calm,  during 
which  the  symptoms  of  the  local  disease,  on  which  they  dépend, 
alone  persist.  But,  in  the  natural  course  of  things,  as  the  affec- 
tion progresses,  the  crises  tend  to  draw  doser,  to  become  con- 
founded,    so  as   to   finally    constitute,  as   it   were,  a  permanent 


2G8  PATHOLOGICAL  ANATOMY. 

vertiginous  state^  in  the  midst  of  which  more  or  less  fréquent 
paroxysms  appear,  and  reproduce  ail  tlie  phenomena  of  the  old 
crises.  The  patient  whom  I  hâve  introduced  to  you  offers  a  very 
marked  example  of  those  suhintrant  crises,  which  it  is  in  some  sort 
customary  to  see,  I  repeat,  in  patients  who  hâve  been  for  years 
suffering  from  the  grave  forms  of  Ménière's  disease. 

You  readily  understand,  gentlemen,  the  use  which  may  be  made 
of  ail  the  éléments  which  hâve  been  hère  put  together  for  the 
elucidation  of  the  diagnosis. 

I  reserve  the  privilège  of  telling  you,  on  another  occasion, 
what  is  most  surely  known  relative  to  the  patkological  anatom,y 
and  to  the  tlieory  of  the  disease.  With  respect  to  the  latter,  the 
experiments  of  Flourens,  MM.  Brown-Scquard,  Vulpian,  Czermak, 
G  ail,  and  Loewenberg,  which  consist  in  reproducing  différent  lésions 
of  the  semicircular  canals  in  animais,  hâve  furnished  important 
data,  as  we  are  aware.  To-day,  I  shall  terminate  by  some  obser- 
vations relative  to  prognosis  and  therapeusis. 

It  is  very  remarkable  to  find  that,  as  a  rule,  the  grave  lésions  of 
the  nervous  centres  which  are  so  frequently  the  conséquence  of 
différent  diseases  of  the  car  do  net  usually  intervene  in  Ménière's 
disease,  even  when  the  latter  has  reached  its  highest  degree  of 
intensity.  This  is  how  matters  most  usually  proceed  in  the  most 
marked  cases  :  deafness  progressively  augments  and,  at  a  given 
moment,  it  becomes  complète,  absolute, 

The  vertiginous  symptoms  and  the  whistling  sounds  proceed,  as 
it  were,  abreast,  in  this  sensé,  namely,  that  they  gradually  diminish 
and  finally  disappear.  It  has  been  so,  for  instance,  in  the  patient 
of  whom  I  spoke  above,  as  having  fallen  in  one  of  his  fits  in  the 
Place  de  la  Bourse.  Subject  to  whistlings,  and  to  vertigos,  since 
1863,  he  is  now  completely  freed  from  them.  But,  on  the  other 
hand,  he  has  become  deaf,  so  deaf  that  although  he  lives  near  the 
Champ  de  Mars,  he  heard  absolutely  nothing  at  the  time  of  the 
explosion  of  the  powder-magazine  in  the  Avenue  Eapp  (187 1).  I 
hâve  often  asked  myself  if  it  would  not  be  proper  to  endeavour  to 
hasten  this  consummation,by  some  intervention,  at  least  in  the  graver 
cases;  for  instance,  when  the  patients  are  reduced  to  the  lamentable 
State  in  which  you  hâve  seen  the  patient  G —  ;  this  is  a  view 
which  I  submit  for  your  considération. 

ITowever  this  may  be,  I  should  not  hide  from  you  that  the 
vertigo  of  Ménière  very  often  resists  the  best  directed  treatment. 


T  RE  ATM  EN  T.  269 

I  have,  however,  several  times  seen  tlie  vertigo,  when  counected  with 
catarrh  of  the  cavity  of  tlie  tympanuin,  improve  and  even  disappear 
under  the  influence  of  the  ordinary  treatment  of  the  latter  affection  ; 
this  is  the  place  to  remind  you  of  the  very  interesting  case  noted 
by  M.  Hillairet,  in  which  the  vertigo  ceased  completely  after  the 
opening  of  an  abscess  in  the  middle  ear. 

The  application  of  energetic  counter-irritation  ought  not  to  be 
neglected  in  severe  cases.  I  will  mention,  in  connection  with  this, 
the  folio wing  fact,  which  I  hâve  recently  witnessed.  One  of  our 
provincial  brethren,  now  aged  44,  experienced,  six  years  ago,  for 
the  first  time,  a  sensé  of  heaviness  in  the  head  and  buzzing  in  the 
ears,  coining  on  in  fits.  Some  months  later,  whilst  driving  alone 
in  the  country,  he  suddenly  felt  intolérable  whistling  in  his  left  ear, 
and  at  the  same  moment  his  head  became  heavy,  and  seemed  to  drag 
him  forward.  He  was  obliged  to  get  down  from  his  carriage,  and 
to  lie  down,  at  full  length,  upon  the  roadside.  Nausea,  foUowed 
by  vomiting  of  glairy  matter  mixed  with  bile,  terminated  the  crisis. 
rits  of  the  same  kind  frequently  recurred  since  that  period,  and, 
at  the  same  time,  the  hearing  power  of  the  affected  ear  diminished. 
Nothing  was  discovered  on  examination,  except  a  certain  degree  of 
thickening  of  the  membrana  tympani.  Ail  the  remédies  a])plied 
having  failed,  I  proposed,  for  want  of  something  better,  tlie  applica- 
tion of  the  actual  cautery,  in  points,  upon  the  left  mastoid  région. 
The  applications  were  repeated  three  or  four  times.  After  this 
treatment  ail  the  symptoms  became  very  distinctly  milder.i 


Gentlemen,  I  thought  it  would  be  interesting  to  inaugurate  the 
conférences  of  this  year"  by  showing  you,  at  the  Salpêtrière — that 
is,  in  an  institution  largely  devoted  to  reputedly  incurable  chronic 
cases — two  examples  of  cure,  or,  at  ail  events,  of  amendment  équi- 
valent or  nearly  équivalent  to  recovery.  The  term  incurable  diseases 
cannot,  of  course,  be  taken  in  an  unqualified  sensé  ;  for  if  it  appHes 
to  cases  which  really  are  beyond  remedy,  it  also  applies  to  those  for 
which  the  remedy  has  not  yet  been,  but  for  which  it  yet  may  be 
found. 

'  Tliis  lecture  was  delivered  in  Jauuary,  1874,  and  first  published  in  'Pro- 
grès Médical,'  Nos.  4  and  5,  1874. 

2  Lecture  delivered  in  November,  1875,  and  published  in  '  Progrès  Médical,' 
No,  50,  1875. 


270  CURE   OP    REPUTEDLY  INCURABLE    CASES. 

The  cases  which  are  about  to  be  introduced  to  your  notice  form 
two  very  distinct  groups  ;  in  one,  the  recovery  or  improvement 
took  place  spontaneously  without  the  intervention  of  the  médical  art  ; 
in  the  other,  it  has  been  intended,  sought  for,  premeditated.  I 
shall  particularly  dwell  upon  a  case  which  belongs  to  the  latter  group. 

This,  gentlemen,  is  the  case  of  a  patient  whom  I  showed  you,  for 
the  first  time,  two  years  ago,  and  whom  I  again  showed  you  last 
year.  I  will  not  discuss  at  any  length  the  clinical  history  of  the 
case  ;  you  can  read  it  in  the  '  Progrès  Médical/^  which  contains  ail 
the  détails.  It  has,  besides,  been  republished  in  most  of  the  French 
and  foreign  médical  journals.  I  shall  confine  myself  to  stating  the 
most  salient  feature  of  this  history,  in  order,  chiefly,  to  enable  you 
to  thoroughly  realise  what  the  condition  of  affairs  was  when  thera- 
peutic  treatment  began. 

It  is,  in  my  opinion,  a  very  fine  example  of  Ménierés  disease, 
or,  better  still,  of  Ilénière's  vertigo  ;  for  the  syndromus  to  which 
thèse  dénominations  relate  do  not  exclusively  answer  to  one  morbid 
state  only  ;  it  may  show  itself  common  to  very  différent  diseases  of 
the  ear. 

The  position  of  affairs  at  this  epoch,  that  is,  in  last  May, 
was  absolutely  the  same  as  in  1874,  that  is  to  say,  that  G —  was, 
for  several  years,  literally  confined  to  bed  under  the  infliuence  of  a 
vertiginous  state,  permanently  established,  so  to  speak,  and  was 
thereby  rendered  nearly  incapable  of  spontaneously  executing  move- 
ments  of  any  extent.  The  slightest  shake  of  the  bed  also  exaspe- 
rated  it,  to  a  high  degree,  and  doubtless  many  of  you  remember  the 
scared  and  anxious  look  of  the  patient's  face,  the  rending  shrieks 
which  she  uttered,  when  she  was  carried  on  a  litter  into  the  lecture 
hall. 

In  addition  to  this  vertiginous  state,  which  was,  in  some  sort, 
habituai,  and  which  is  accompanied  by  an  incessant  rustling  heard 
in  the  ear,  G —  was  subject  to  the  occurrence  of  great  vertiginous 
crises,  from  time  to  time.  I  dwelt  much,  on  a  former  occasion,  on 
the  description  of  thèse  crises,  because  I  believe  they  constitute  the 
fundamental  symptomatic  fact,  in  Ménière^s  disease,  whilst  the 
habituai  vertiginous  state  may,  in  some  sort,  be  considered  as  a 
complication,  an  epiphenomenon  which  only  shows  itself  in  excep- 
tional  cases. 

The  fits  in  question  are,  as  you  recollect,  alvvays  heralded  and 

•  1874,  Nos.  4  and  5.    M.  Charcot  bere  refers  to  the  precedhig  lecture. 


TEEATMENT.  271 

immediately  précédée!  by  tlie  sensation  of  a  shrill  piereing  souiid, 
which  supervenes  suddenly  and  unexpectedly,  and  wliicli,  in  the  case 
of  G —  in  particular — as  well  as  in  many  other  patients  of  the 
same  kind — simulâtes,  it  seems,  the  strident  sound  of  a  railway 
whistle  so  closely  as  that  it  might  be  mistaken  for  it.  I  hâve  been 
led  to  consider  this  abrupt  whistle  as  one  of  the  constant  necessary 
éléments,  and,  consequently,  as  a  characteristic  of  vertigo  ah  mire 
lœsd.  Qaite  recently,  Dr.  Lussana,  in  a  séries  of  interesting  articles 
published  in  the  'Gazetta  Medica  Italiana  Lombardia'  (1875, 
t.  XXXV,  ser.  vii,  t.  ii,  No.  43,  et  seq.),  has  confirmed  this  point, 
which  is  of  interest,  both  to  clinical  art  and  to  pathological 
physiology. 

As  to  the  vertigo  itself,  it  shows  itself,  in  G — ,  conformable  to 
the  classic  type  ;  after  the  whistle  a  feeling  of  abrupt  translation 
movement,  in  conséquence  of  which  the  patient  imagines  herself  flung 
forward  or  backward,  as  if  by  the  impulse  of  some  external  force. 
Latéral  impulses  are  much  rarer  in  lier  case.  Then  we  note  absence 
of  loss  of  consciousness,  and  présence  of  nausea  and  sometimes 
vomiting  at  the  end  of  the  fit. 

I  would  remind  you,  as  we  pass,  that  this  sort  of  motor  halluci- 
nation is  not  always  Platonic,  if  you  will  excuse  the  word  ;  I  hâve 
quoted  several  cases  where  the  patients  actually  impelled  by  the 
vertigo  fell  heavily  on  the  face;  one  of  them  broke  the  nasal  bones  : 
another,  of  whom  I  shall  soon  speak,  broke  several  teeth. 

I  will  conclude  by  remarking  that,  in  the  case  of  G — ,  the  dis- 
ease  is  of  very  old  standing,  the  first  symptoms  dating  back  over 
ten  yearsj  that  there  exists  in  the  left  side  a  lésion  of  the  drum 
with  thickening  of  the  membrana  tympani,  and  an  habituai  running 
of  pus  mingled  with  blood. 

Now,  that  you  hâve  présent  to  the  mind  the  former  symptom- 
picture,  I  shall  enter  into  some  détails  to  show  you  under  what 
circumstances  the  improvement  was  effected  which  has  enabled  G — 
to  stand  upright,  as  you  perceive,  and  to  walk  without  assistance  ; 
to  spend  the  day  tranquilly  seated  in  an  arm-chair  and  to  endure, 
without  much  émotion,  ail  the  shaking  and.  shocks  to  which 
curiosity  may  subject  her. 

At  the  time  of  my  conférence  on  the  vertigo  of  Ménière,  de- 
delivered  in  1874,  I  stated  some  therapeutic  considérations. 
Amongst  others,  I  noticed  that  vertigo,  ab  aure  lœsâ,  even  when 
most  inveterate,  recovered  sometimes    spontàneously,   when    the 


272  TEEATMENT  BY  QUININE. 

patient  became  completely  and  irreparably  deaf,  and  ceased  to  liear 
the  whistling.  1  also  asked  myself  if  it  miglit  not  be  possible  to 
purposely  obtain  this  often  désirable  resuit  by  surgical  interven- 
tion. I  also  recommended  tlie  employment  of  cautery  in  points 
over  the  mastoid  processes.  The  method  which  I  did  employ  in 
the  case  to  which  I  invite  your  attention,  is  both  much  simpler 
and  much  less  radical. 

ïhe  idea  occurred  to  me  that,  by  means  of  sulphate  of  quinine, 
which  as  every  one  knows,  détermines,  amongst  other  phenomena, 
more  or  less  marked  rustling  and  buzzing  in  the  ear,  we  might 
perhaps  succeed  in  producing  lasting  modifications  in  the  action  of 
the  auditory  nerve,  by  sufficiently  prolonging  the  employment  of 
large  doses.  The  resuit,  as  you  will  see,  has  justified  thèse  antici- 
pations. 

Giraud  has  taken  sulphate  of  quinine,  in  doses  of  from  0*50 
centigrammes  to  i  gramme  daily,  in  a  regular  manner — excepting 
several  interruptions  of  some  days,  rendered  necessary  by  gastric 
pains — during  the  whole  of  the  months  of  May,  June,  and  the  first 
twenty  days  of  July. 

Towards  the  beginning  of  June,  that  is  to  say,  about  four  weeks 
after  the  commencement,  it  was  remarked  that  for  some  days  the 
patient  might  be  shaken  in  lier  bed,  and  transferred  from  one  bed 
to  another  without  giving  utterance  to  any  cry  of  distress,  as  had 
been  once  constantly  the  case  under  sucli  circumstances. 

When  interrogated,  she  informed  us  that  the  shrill  whistling  had, 
towards  the  same  epoch,  first  diminished  in  intensity  and  then 
completely  ceased,  simultaneously  with  the  great  vertiginous  crises. 
The  permanent  buzzing  had  been  replaced  by  a  rustling,  quite 
différent  in  character,  which  seems  to  be  connected  with  the  action 
of  the  quinine.  Encouraged  by  thèse  first  results,  I  requested  G — 
to  rise,  and  to  try  to  stand  and  walk.  She,  at  first,  refused  ener- 
getically,  dreading  the  return  of  the  vertigo,  and  also  judgiug  the 
attempt  impracticable,  to  say  no  more.  I  insisted  ;  finally,  she 
consented  and,  one  fine  day,  aided  by  two  assistants  she  succeeded 
in  making,  not  without  great  effort,  two  or  three  steps,  rendered 
very  difficult  owing,  it  appears  to  excessive  sensitiveness  of  the 
soles  of  the  feet  rather  than  to  any  vertiginous  state.  I  required 
the  experiment  to  be  repeated  every  day.  Bapid  progress  was 
made,  for,  about  the  2oth  June  (when  the  sulphate  of  quinine  was 
suspended)  G —  had  already  been  able  several  times,  with  sufiîci- 


ANOTHEE    CASE.  273 

entlj  firm  gait  and  no  other  help  than  lier  stick,  to  walk  arouud 
the  inuer  boundary  of  tliis  vast  hospital.  The  disease^  sinoe  that 
period  has  made  no  aggressive  return,  and  y  ou  may  judge  by  your- 
selves  that  the  attitude  of  the  body  and  the  gait  do  not  much 
diverge  from  those  of  a  person  in  good  health.  I  would  add  that 
jshe  is  neither  more  nor  less  deaf  than  before.  Hence,  as  you  see, 
the  sulphate  of  quinine  has  not  operated  hère  by  determining  com- 
plète paralysis  of  the  auditory  nerve. 

The  case  to  which  I  hâve  just  called  your  attention  is  not  unique 
of  its  kind  ;  I  could  mention  others  where  the  happy  influence  of 
the  prolonged  use  of  sulphate  of  quinine  has  been  reported.  I 
shall  confine  myself  to  one  of  them,  because  the  resuit  obtained 
was  verified  by  several  of  my  professional  brethren. 

I  was  called,  in  June,  1875,  to  give  my  advice  concerning 
the  state  of  a  lady,  aged  about  30,  who,  for  the  past  eighteen 
months,  had  become  subject  to  epileptiform  crises,  it  was  said,  or 
at  ail  events  to  hystero- epileptiform  fits.  The  bromide  of  potas- 
sium had  completely  failed. 

At  the  consultation,  it  was  thoroughly  established  that  neither 
syphilis,  nor  hysteria,  nor  any  traumatic  cause  vrhatever  played  a 
part  in  her  antécédents.  The  patient,  a  very  intelligent  lady,  at  my 
request,  willingly  entered  into  the  most  minute  détails  concerning 
the  character  of  her  fits.  I  learned  from  her  that,  during  thèse 
attacks,  she  was  greatly  disturbed,  greatly  agitated,  undoubtedly, 
but  had  never  lost  consciousness.  The  onset  had  always  been 
abrupt  and  unexpected.  The  sensation  of  falling  forward  came  on 
suddenly  and,  in  point  of  fact,  she  had  been  actually  flung  forward 
several  times,  with  her  face  to  the  ground,  and,  once,  two  of  lier 
teetli  were  broken.  Hence,  we  had  hère  a  vertigo  of  translation, 
with  abrupt  onset,  antero-posterior  falling,  no  loss  of  consciousness, 
and  therefore,  some  of  the  most  marked  characters  of  the  vertigo 
of  Ménière.  In  order  to  complète  the  resemblance  I  will  add  that, 
in  the  case  of  Madame  X — ,  the  fit  often  concluded  by  nauseas,  or 
even  by  vomiting. 

Thèse  preliminary  data  once  obtained,  I  summarily  explored  the 
state  of  the  auditory  acuteness,  by  the  help  of  a  watch,  and  I  found 
that  the  hearing  was  very  manifestly  weakened  on  the  left  side. 
Then,  I  asked  a  final  question.  The  reply  to  this  should,  in  my 
opinion,  décide  the  matter.  I  enquired  whether  it  did  not  some- 
times  happen  that  a  shrill  sound  more  or  less  analogous  tothat  of  a 

VOL.   II.  18 


274  ANOTHER    CASE. 

whistle  was  heard  in  the  ears.  The  husband,  who  was  présent  at 
the  consultation,  now  spoke,  quickly  replying  that  Madame  X — , 
in  fact,  was  often  surprised  to  liear  the  whistle  of  locomotives  from 
a  neighbouring  railway  station  at  unusual  hours,  whilst  nothing 
of  the  kind  had,  in  reality,  occurred.  The  connexion  between  the 
abrupt  development  of  whistling  sounds  and  the  invasion  of  the 
vertiginous  fits  was,  after  this,  readily  established. 

I  believed  I  could  announce  that  we  had  hère  neither  epilepsy, 
nor  hystero-epilepsy,  but  Ménière's  vertigo.  I  gave  hopes  that,  by 
the  assistance  of  an  appropriate  treatment,  the  fits  might  be  made 
milder,  and  perhaps  suppressed.  Sulphate  of  quinine  was  pre- 
scribed  in  doses  of  60  centigrammes,  and  the  employment  of  this 
dose  was  to  be  continued  for  two  months.  Shortly  after  the  com- 
mencement of  the  treatment,  the  whistling,  and,  simultaneously,  the 
vertiginous  fits  ceased  to  be  experienced.  They  hâve  not  made 
their  appearance  since.^ 

*  I  could,  to-day,  quote  a  good  number  of  other  examples  where  the  ver- 
tiginous phenomena,  in  question,  bave  been  very  remarkably  amended  or  even 
completely  cured  by  the  influence  of  the  'prolongea  use  of  sulpliate  of  quinine. 
Dr.  Weir  Mitcheli  related  several  facts  of  this  kind,  at  the  last  congress  at 
New  York. 


LECTURE  XIX. 
ON  POST-HEMIPLEGIC  HEMICHOREA. 

SuMMARY. — Post-hemiplegic  Iiemichorea.  Clinicalfacts.  Dlsorders 
qf  gênerai  and  spécial  sensïhïltty.  Motor  disorders  :  their 
resemblance  to  ckoreic  movements.  Trépidation  of  hémiplégie 
patients.  Instahility  of  members,  ajfeded  bi/  post-hemiplegio 
hemichorea.  Characters  of  muscular  disorders  in  rest  and  in 
motion. 

Organic  lésions,     Foci  of  intra-encephalic  Jiœmorrhage  and 
ramollissement  ; — partial  atropTiy  of  tke  hrain. 

Rarity    of  post-hemip/legic  hernicliorea.      Relation  between 

hemichorea  and  hemianœsthesia.      Seat  of  lésions  to  which 

•     thèse  symptoms  belong  ;  posterior  extremity  ofthe  optic  thalami  ; 

posterior  portion  of  the  caudale  nucleus  ;  posterior  portion  of 

the  corona  radiata. 

Pra-hemiplegic  hemichorea. 

Gentlemen^ — I  will,  this  morning,  ask  your  most  particular 
attention  to  a  group  of  phenomena  which  I  propose  to  designate 
by  the  name  of  post-hemiplegic  hemichorea.  This  dénomination  I 
borrow  from  Dr.  S.  Weir  Mitchel,  of  Philadelphia,  who  has  made 
use  of  it  in  a  récent  work.^  Those  amongst  you  who  hâve  followed 
ray  lectures,  during  the  last  few  years,  will  easily  recognise  this 
morbid  state,  interesting  examples  of  which  I  hâve,  on  différent 
occasions^  exhibited  to  them.^ 

According  to  the  line  of  conduct,  which  I  traced  for  myself, 
in  resuming  thèse  clinical  lectures — which  I  would  in  some  sort 
compare  to   the  "  object-lessons/'  so  widely  adopted  in  American 

'  "  Post-paraljtic  Chorea,"  in  the  'American  Journal  of  the  Médical  Sciences/ 
Oct.,  1874,  342. 

^  Leçons  cliniques  sur  les  maladies  du  Système  Nerveux,'  t.  I,  1872-73, 
p.  279. 


276  CLINICAL    FACTS. 

scllools — I  shall  do  my  best  to  form  a  clear  and  Ml  picture  of 
post-hemiplegic  hemichorea  out  of  the  history  of  tlie  three  patients 
whom  I  am  about  to  question,  in  your  présence. 

The  first  patient,  R —  Marie,  is  fifty-one  years  of  âge.  In  lier 
antécédents  which,  on  the  whole,  possess  but  a  médiocre  interest,  I 
will  only  notice  the  foUowing  points  :  the  patient  had  typhoid  fever  at 
i8,  and  at  30,  she  had  intermittent  fever  which  lasted  six  months. 

At  41,  E —  got  an  apoplectic  attack,  with  loss  of  consciousness, 
and  accompanied,  it  appears,  with  vomiting.  Having  regained  her 
sensés,  she  presented  a  hemiplegia  with  complète  flaccidness  of  the 
right  side.  During  the  six  months  that  followed  the  patient  had, 
as  she  déclares,  an  exact  notion  of  objects.  She  remembered  their 
names — consequently  she  had  no  verbal  amnesia — but  she  was  in- 
capable of  pronouncing  them,  owing  to  a  difficulty  in  the  articulation 
ofwords.  I  will  add,  moreover,  that  during  this  same  period,  there 
was  momentarily,  if  we  are  to  accept  her  statement,  a  certain  degree 
of  contracture  in  the  muscles  of  the  right  hand. 

However  this  may  be,  at  the  end  of  thèse  six  months,  R —  was  able 
to  walk  more  or  less  well.  But,  whilst  she  little  by  little  and  pro- 
gressively  recovered  motion  in  the  right  lower  limb,  the  movements 
of  the  corresponding  upper  extremity,  although  they  had  becorae 
possible  and  even  extensively  so,  were  soon  embarrased  by  a  trem- 
bling  of  a  spécial  kind,  choreic  in  character  : — it  is  on  this  trembling 
that  I  am  about  particularly  to  insist. 

Matters  continued  in  the  same  way  since  that  period,  that  is  to  say, 
for  about  ten  years,  and,  especially,  during  the  five  years  which  hâve 
just  elapsed,  and  during  which  R —  has  not  ceased  to  be  underour 
observation  at  the  Salpêtrière. 

We  hâve  to  consider,  in  its  présent  condition,  the  state  of  the 
right  side  of  the  body,  first  as  regards  the  sensibility,  then  with 
respect  to  the  motor  functions.  I  show  you,  in  the  first  place,  the 
patient  reposing  on  her  couch,  in  order  to  facilitate  our  investiga- 
tions ;  but  I  will,  in  a  few  minutes,  show  her  to  you  in  the  vertical 
position,  and  whilst  walking. 

a.  There  exists  in  R — ^s  case  a  gênerai  hemianœsthesia  of  the 
right  side  of  the  body.  In  other  terms,  sensibility  is,  on  this  side, 
gravely  altered  in  the  face,  trunk,  and  members.  Thus  far  with 
respect  to  gênerai  sensibility.  This  is  not  ail  ;  hearing,  tante,  and 
the  true  cephalic  sensés  :  smell  and  vision  are  duUed  ou  the  same 
side.     Prom  this  point  of  view  we  shall  find  in  this  patient,  as  you 


DISORDEES    OF    SENSATION.  277 

have  guessed,  an  exact  reproduction  of  the  characters  of  hysterical 
hemiansesthesia  whicli  are  so  well  known  to  you. 

Thus  :  1°.  As  regards  vision,  there  is,  in  the  right  eye,  a  modifica- 
tion, though  a  slight  one  ;  visual  acuity  is  hère  simply  diminished. 
2°.  With  respect  to  smell,  the  altération  is  more  marked;  the 
patient  is  absolutely  unabie  to  perceive,  with  the  right  nostri],  the 
odour  of  ether.  3°.  The  change  is  still  more  distinct  where  the 
hearing  is  concerned  :  the  patient  who  hears  very  distinctly  the 
ticking  of  a  watch  with  the  left  ear  only  perceives  it  in  a  very  con- 
fused  manner  with  the  right.  This  diminution  of  hearing  power 
was  noted  from  the  commencement  of  the  observation,  for  R — com- 
plained  of  it,  herself,  at  the  outset,  without  having  had  her  attention 
called  to  this  point.  4°.  The  loss  of  taste  will  be  placed  beyond 
question  by  the  test  with  colocynth  which  we  are  going  to  apply. 
You  see  that  she  does  not  perceive  its  bitterness  on  the  right, 
whilst  she  denounces  it  the  moment  the  substance  touches  the  left 
side  of  the  lingual  mucous  membrane. 

You  havejust  verified,  by  yourselves,  gentlemen,  the  exactness 
of  our  assertion  so  far  as  the  spécial  sensés  are  concerned  ;  you  will 
be  convinced,  in  an  instant,  that  the  examination  will  not  be  less 
conciusive  when  it  bears  on  the  question  of  gênerai  sensibility  :  the 
latter  is,  I  repeat,  manifestîy  diminished  over  an  entire  half  of  the 
body.  The  contact  of  a  cold  substance  is  much  more  keenly  felt 
on  the  left  than  on  the  right.  Tickling,  introduction  of  a  foreign 
body  into  the  right  nostril,  scarcely  produces  any  reaction.  Lastly, 
over  ail  this  side  of  the  body  :  face,  trunk,  and  limbs,  an  analgesia 
of  the  most  marked  character  exists,  since  you  can  plunge,  deeplj 
and  rudely,  a  large  pin  into  thèse  parts  without  the  patient  showing 
the  slightest  sign  of  suffering. 

This,  gentlemen,  as  I  reminded  you  a  few  months  ago,  is  an 
union  of  symptoms  which  we  have  often  occasion  to  observe  in 
hysteria,  and  more  especially  in  ovarian  hysteria. 

But,  in  the  case  of  E — ,  hysteria  is,  however,  not  in  any  way  at 
work,  an  additional  proof,  if  need  were,  that  in  diseases  of  the 
nervous  system,  as  in  ail  others,  no  phenomenon,  taken  singly,  can 
be  truly  characteristic.  It  is  the  mode  of  groupiag  of  the  ])he- 
nomena,  their  mode  of  évolution,  concaténation,  the  whole  array  of 
circumstances  which  chiefly  serve,  hère  as  elsewhere,  to  establish 
nosographic  distinctions, 

h.  I  now  approach  the  second  point,  to  wit  :  the  study  of  the 


278  MOTOR    DISORDEES. 

wotor  disorders  whicli  tliis  woman  présents.  Thèse  disorders  are 
very  peculiar.  If  tliey  be  déficient  in  the  face,  in  this  instance, 
thej  are,  on  the  contrary,  very  évident  in  the  upper  and  lower 
extremities  of  the  right  side.  Thèse,  as  I  can  show  you  at  once, 
présent  neither  atrophy  nor  contracture,  nor  any  deformity  what- 
ever.  We  will  now  examine  them  successively,  when  at  rest,  and 
when  in  motion. 

In  intentional  movements  of  the  upper  limb  a  choreiform  agitation 
is  manifested  which  is  altogether  comparable  to  that  which  I  hâve, 
on  a  former  occasion,  described  in  connection  with  disseminated 
sclerosis.  So  long  as  R —  is  quiet,  at  rest,  there  is  almost  no 
motor  disorder  in  the  limb  ;  on  the  other  hand,  in  the  act  of  lifting 
a  glass  of  water  to  the  lips,  the  arm  is  immediately  seized  with  very 
extensive  rhythmical  movement,  so  that,  if  she  were  not  watched, 
the  water  would  be  violently  dashed  about  in  ail  directions.  We 
shall  see,  in  one  moment,  when  she  is  walking,  analogous  pheno- 
mena  exhibited  in  connection  with  the  right  lower  limbs. 

This  motor-disorder  not  only  resembles  the  trembling  of  dis- 
seminated sclerosis,  asi  hâve  just  said,  but  italso,  in  some  respects, 
resembles  a  phenomenon  which  is  ordinarily  observed  in  common 
cases  of  hemiplegia;  I  allude  to  the  tremulation  which  rarely  fails  to 
appear  wheu  the  latéral  columns  of  the  cord  are  afFected  with  sclerosis 
to  a  certain  degree,  provided  always  that,  contracture  being  little 
marked,  the  voluntary  movements  are  still  possible  to  a  certain 
extent.  Thèse  post-hemiplegic  choreiform  movements  diverge,  on 
the  other  hand,  from  the  incoordination  of  ataxic  patients,  were  it 
only  because  of  the  fact  that  vision  exerts  no  influence  over  them. 

But,  there  is  a  character  which  fundamentally  divides  thèse  cho- 
reiform movements  from  the  trembling  of  disseminated  sclerosis  ; 
the  tremulation  of  hémiplégie  patients,  and  the  motor  incoordina- 
tion of  ataxia  ;  and  which,  on  the  other  hand,  brings  them  close  to 
chorea;  this  is  the  existence  of  an  indahïlity  cf  the  affected  memhers 
at  a  time  when  the  patient  does  not  will  any  movement.  The 
limbs,  even  in  this  case — it  is  ver}/  important  to  remark — are 
stirred  by  small  involuntary  movements  analogous  to  those  which, 
in  the  same  circumstances,  are  seen  in  ordinary  choiera.  Thèse 
movements,  as  you  may  observe,  are  very  manifest  in  the  lower 
extremity.  You  see  in  fact,  the  patella,  as  it  were,  incessantly 
raised,  and  that  in  a  rhythmical  manner,  by  the  suddcn  and 
involuntary  contractions  of  the  anterior  muscles  of  the  thigli  ;  you 


CHOKEIFOEM    CHAEAOTER    OF   MOVEMENTS.  279 

see^  at  the  same  time,  the  foot^  in  spite  of  the  patientas  will, 
twisted  by  turns  into  adduction  and  abduction,  and  tlien  liexed  and 
extended. 

The  handj  as  is  also  seen,  cannot  remain  at  rest,  by  the  patientas 
side;  it  is  constantly  shakeu  by  sudden  and  unexpected  jerks,  whilst, 
at  the  same  time,  the  fingers  are  stretched  or  flexed,  without  cause. 

I  will  add  that,  in  cases  absolutely  comparable  to  the  preceding, 
but  still  more  marked,  thèse  involuntary  and  very  extensive  move- 
ments  constitute  a  perpétuai  agitation  which,  in  fact^  causes  this 
state,  so  far  as  the  character  of  its  motor  disorders  is  concerned, 
to  differ  in  no  essential  features  from  chorea  properly  so  called. 

Hence,  it  is  to  the  term  cJiorea  that  we  shall  hâve  recourse  in 
order  to  designate  the  phenomenon  in  question.  Of  course  we 
wish  to  indicate  a  simple  resemblance,  and  by  no  means  a  real 
nosographic  assimilation  witli  common  chorea  [chorea  minor)  ; 
undoubtedly  the  habituai  coexistence  of  hemianeesthesia,  long  since 
noticed  in  ordinary  chorea,  according  to  the  observations  of  my 
former  colleague,  Moynier,  is  also  a  trait  which  the  latter  possesses 
in  common  with  the  disease  which  occupies  us  ;  but,  on  the  other 
hand,  distinguishing  characters  abound.  It  will  be  enough  for  me 
to  point  eut,  as  regards  cases  of  post-paralytic  hemickorea,  the 
exact  and  definite  limitation  of  the  motor  disorders  to  one  side  of  the 
body  only  :  next,  the  pre-existence  of  a  long  established  hemiplegia 
with  flaccidness  of  the  muscles  at  first,  then  marked  by  a  certain 
degree  of  contracture,  finally  the  sudden  and  truly  apoplectic  onset 
of  the  phenomena.  Thèse,  you  see,  are  phenomena  which  do  not 
belong  to  the  common  St.  Yitus's  dance. 

In  short,  the  affection  from  the  beginning,  showed  itself  in  R — 's 
case,  under  the  form  of  cérébral  apoplexy  foUowed  by  abrupt  for- 
mation of  a  focus  of  intra-encephalic  ramollissement  or  haemor- 
rhage.  And  in  reality,  gentlemen,  it  is,  beyond  a  doubt,  to  one  or 
other  of  thèse  organic  lésions  that  we  must  refer  the  phenomena 
which  I  hâve  pointed  out  in  oui  patientas  case. 

The  choreiform  movements,  of  which  I  hâve  striven  to  make  you 
acquainted  with  the  principal  characters,  présent  themselves  under 
a  new  aspect,  when  the  patient  endeavours,  with  the  help  of  a  stick 
which  she  holds  in  lier  left  hand,  to  stand  upright  and  motionless, 
or  proceeds  to  walk.  Then  the  whole  body,  as  you  see,  is  shaken 
with  jerks  which  resuit  from  the  fact  that  successive  movements  of 
sudden  flexion  and  extension  take  place,  involuntarily,  in  the  knee, 


280         PARTIAL  ATEOPHY  OF  THE  BRAIN. 

and  in  the  ankle-joint  on  tlie  right  side.  You  will  remark  that,  on 
the  contrary,  the  upper  limb  of  the  same  side  remains  nearly 
motionless.  But  that  happens  simply  because  of  a  subterfuge  ; 
the  hand  is^  in  fact,  held  closely  applied  along  the  side  of  the  body, 
or  else  thrust  into  a  pocket  ;  without  which,  it  would,  like  the  lower 
extremity,  be  constantly  in  motion. 

Post-hemiplegic  hemichorea  does  not  exhibit  itself  connected 
only  with  the  existence  of  foci  of  intra-encephahc  hsemorrhage, 
and  ramollissement,  such  as  we  commonly  see  in  the  adult.  It 
may  also  supervene,  owing  to  those  lésions,  still  rather  imperfectly 
known,  at  least  in  the  first  phases  of  their  development,  which,  in 
young  children,  détermine  what  is  termed  partial  ai,rophj  of  the 
hrain  (Cotard,  'Thèse  de  Paris,'  j868).  The  usual  conséquence 
of  thèse  altérations,  as  Bouchet  and  Cazauveilh  hâve  long  since 
shown,  is  an  incurable  hemiplegia,  most  frequently  with  contrac- 
ture (spasmodic  hemiplegia  of  Heine).  But,  in  such  a  case,  it  may 
occur,  though,  indeed,  very  exceptionally,  that  the  hemiplegia  gives 
place,  as  it  were,  from  the  outset  to  a  hemichorea,  quite  similar  to 
that  which  we  hâve  just  described.  When  once  constituted,  this 
hemichorea  will  persist  so  long  as  life  lasts.  I  am  in  a  position  to 
place  two  examples  of  this  kind  before  your  eyes. 

R —  is,  at  présent,  aged  i8.  Placed  soon,  after  lier  birth,  in 
the  country,  she  seems  to  hâve  been  subject  to  convulsions  since 
she  was  two  years  old  ;  it  is  certain  that  when  she  was  taken  home, 
by  her  parents,  at  the  âge  of  four  and  a  half  years,  the  upper  and 
lower  extremities,  on  the  right  side,  were  paralysed,  and  that  she 
got  epileptic  fits  from  time  to  time.  Her  gênerai  health  was,  more- 
over,  much  affected,  and  she  remained  constantly  sitting  or  lying 
down.  Thanks  to  the  care  lavished  on  her,  she  gradually  recovered 
strength,  and  even  at  the  end  of  some  months  became  able  to  walk 
and  to  make  some  use  of  her  right  arm.  From  this  moment,  it 
was  remarked  that  the  right  hand  was  agitated  by  a  sort  of  trem- 
bling,  during  intentional  motion  ;  but  the  choreiform  movements, 
supervening  independently  of  any  voluntary  act,  were,  it  appears, 
chiefly  marked  from  the  time  she  was  seven  years  of  âge.  They  bave 
not  ceased  to  exist  since  that  period.  I  shall  not  enter  into  détails 
respecting  them.  That  would  only  be  to  reproduce  every  point  of 
the  description  given  in  référence  to  our  first  patient.  I  will  only 
point  out  that,  contrary  to  what  takes  place  in  the  great  majority 
of  cases  of  post-hemvplegïc  hemichorea  of  the  adult,  hemiansesthesia 


ANATOMICAL    CONDITIONS    OF    HEMICHOEEA.  281 

is  hère  completely  déficient.^  This  very  peculiarity,  tliat  is  to  say, 
the  absence  of  ansesthesia  in  the  limbs  attacked  with  chorea,  was 
also  exhibited  in  the  follouing  case,  wliich  also  relates,  like  the 
foregoing  one,  to  hemiplegia  of  young  infants.^ 

Gr — ,  aged  29,  experienced  at  the  âge  of  eight  months  con- 
vulsions qualified  as  epileptiform,  and  followed  by  hemiplegia 
of  the  left  side.  Since  that  time  she  has  not  ceased  to  be  subject 
to  epileptic  fits.  The  limbs  on  the  right  side  are,  at  présent, 
somewhat  weaker  and  more  slender  than  those  of  the  other  side, 
but  they  are  neither  contractured  nor  insensible.  They  appear 
incessantly  shaken  with  choreiform  movements,  in  which  the  face 
does  not  seem  to  participate,  and  which  are  exaggerated  by  the 
performance  of  intentional  acts. 

I  return  now  to  the  case  of  Ronc — .  The  motor  disorders, 
which  we  hâve  studied  in  this  patient,  are  far  from  being  a  common- 
place  phenomenon  in  the  history  of  intra-encephalic  hsemorrhage 
and  partial  ramollissement  of  the  brain.  In  fact,  in  a  considérable 
number  of  cases,  relating  to  thèse  lésions,  which  I  hâve  collected 
at  the  Salpêtrière  during  a  dozen  years,  I  hâve  remarked  post- 
hemiplegic  hemichorea  five  or  six  times  at  most.  As  a  rule,  when 
hemiplegia  supervenes,  owing  to  the  formation  of  an  intra- cérébral 
focus  of  hsemorrhage  or  ramollissement,  motor  paralysis,  if  the 
case  be  favorable,  gradually  lessens  and  finally  disappears  com- 
pletely without  the  appearance  of  choreiform  movements  at  any 
period  ;  or  else,  if  the  case  be  grave,  the  paralysis  persists  such  as 
it  was,  with  or  without  the  accompaniment  of  permanent  contrac- 
ture, sometimes  complète,  sometimes  incomplète.  Hère,  again — I 
speak,  of  course,  of  the  rule,  and  I  reserve  the  chapter  of  anoma- 
lies— the  choreic  shaking  is  entirely  déficient  ;  only,  if  motor  inertia 
be  incomplète,  it  may  happen,  principally  when  there  is  a  certain 
degree  of  contracture,  that  the  intentional  movements  are  dis- 
turbed  by  a  kind  of  trépidation,  which  we  hâve  already  discussed, 
and  which  has  nothing  in  common,  I  told  you,  with  the  convulsive 
shaking  seen  to  be  manifested  in  chorea,  even  irrespective  of  the 
performance  of  voluntary  acts. 

What,  then,  are  the  apparently  very  spécial  conditions  which, 

•  Permanent  hemiansesthesia  is,  sometimes,  produced  in  conséquence  of 
partial  atrophy  of  the  brain,  dating  from  early  childbood  :  I  hâve  recently  met 
with  au  example  of  this  kind,  in  the  infirmaries  of  the  Salpêtrière. 

ïhis  patient,  and  the  next  mentioned,  belong  to  M.  Dclasiauve's  wards. 


282  ANATOMIOAL    CONDITIONS    OP    HEMICHOEEA. 

in  some  exceptional  cases  of  cérébral  hsemorrhage  or  ramollisement 
{en  foyer'),  cause  tlie  liemiplegia,  contrary  to  the  usual  rule,  to  be, 
at  a  given  moment^  replacée!  by  liemicliorea  ?  I  cannot,  at  présent, 
answer  this  question  by  a  regular  solution.  Hère,  however,  in 
my  opinion,  is  the  direction  in  which  tliis  may  be  sought  for.  I 
believe  that  thèse  foci  of  hsemorrhage  or  encephalomalacia  which 
détermine  hemichorea  assume,  in  the  encephalon,  a  peculiar,  fixed 
position,  quite  différent  from  the  very  varied  positions  occupied  by 
the  foci  which  produce  common  hemiplegia.  I  base  my  opinion 
chiefly  on  this  remarkable  circumstance,  already  made  prominent, 
namely,  that  cerelral  hemimiœsthesia  (that  is,  with  participation  of 
ail  the  spécial  sensés,  including  vision  and  smell) — this  phenomenon, 
which  is  so  rarely  seen  connected  with  common  hemiplegia — is,  on 
the  contrary,  a  very  usual,  though  indeed  not  a  necessary,  accom- 
paniment  of  post-hemiplegïe  hemichorea.  Now,  it  appears  to  be 
established  that  this  particular  form  of  hemianœsthesia  dépends  on 
lésions  localised  in  certain  points,  ever  the  same,  of  the  cérébral  hémi- 
sphères, the  seat  of  which  seems,  to-day,  to  be  well  nigh  ascertained. 
It  is  therefore  already  probable  from  this,  that  the  nerve  éléments, 
fibre-fascicles  or  ganglionic  corpuscles,  the  lésion  of  which  will 
produce  hemichorea,  border  on  those  whose  destruction  détermines 
liemiansesthesia. 

Necroscopy,  besides,  has  testified  in  favour  of  this  hypothesis. 
Three  times,  I  hâve  had  occasion  to  make  the  autopsy  of  subjects,  in 
whom  hemichorea  of  several  years'  standing  had  followed  on  hemi- 
plegia, marked  by  an  abrupt,  apoplectic  onset.  In  thèse  three 
cases,  the  hemiansesthesia  existed,  well  marked,  as  \ve  hâve  seen  in 
the  case  of  our  patient,  Eonc- — .  In  addition,  just  as  in  lier  case, 
but  undoubtedly  owing  to  a  mère  chance  coïncidence,  it  is  the  left 
side  which  is  hère  affected.  However  it  be,  the  lésion  revealed,  on 
autopsy,  consisted  of  ochreoiis  cicatrices,  unmistakable  vestiges  of 
the  former  existence  of  hsemorrhagic  foci.  The  cicatrices  in  ques- 
tion occupied,  in  the  right  hémisphère,  always  the  same  région,  or 
very  nearly  so,  and  hère  is  a  note  of  the  parts  which  they  involved  : 
thèse  were  constantly,  that  is  to  say,  in  ail  the  three  cases  :  i°. 
The  posterior  extremity  of  the  optic  thalamus.  2°.  The  most  pos- 
terior  part  of  the  nucleus  caudatus.  It  is  expressly  noted  that  the 
anterior  two  thirds  or  three  fourths  of  thèse  grey  nuclei  had 
remained  perfectly  sound.  3°.  Lastly,  the  most  posterior  portion 
of  the  crus  of  the  corona  radiata. 


PBiE-HEMIPLEGIC    HEMICHOREA.  283 

In  two  of  the  cases  only,  one  of  the  tuberculi  quadrigemini,  the 
foremost  one  on  the  side  corresponding  to  the  ochreous  focuSj  par- 
ticipated  in  the  altération. 

What,  in  this  enumeration,  are  the  lésions  which  determined 
hemichorea  ;  what,  on  the  other  hand,  are  those  to  which  the  hemi- 
ansesthesia  is  due  ?  The  latter,  as  we  hâve  endeavoured  to  show 
elsewhere,  dépends  on  an  altération  of  the  most  posterior  fascicles 
of  the  corona  radiata.  The  altération  of  the  hindermost  portion 
of  the  optic  thalamus,  that  of  the  tail  of  the  corpus  striatum,  should 
therefore  remain  to  the  crédit  of  the  hemichorea,  for  we  cannot 
rely  on  the  non-constant  lésion  of  the  tuberculi  quadrigemini. 
But,  on  the  other  hand,  we  hâve  often  and  often  seen  the  optic 
thalamus  and  the  lenticular  nucleus  affected  in  their  several  parts 
with  the  most  varied  lésions,  and  not  the  slighest  trace  of  choreic 
movements  resulting.  So  that,  according  to  ail  appearance,  neither 
should  thèse  organs  be  arraigned  in  the  présent  instance.  I  believe 
it  more  probable,  (but  this  is  simply  an  hypothesis  which  I  submit 
for  your  considération  and  criticism),  that  on  one  side,  undoubtedly 
in  front  of  the  fibres  which,  in  the  corona  radiata,  serve  for  the  transit 
of  sensory  impressions,  there  are  fascicles  of  fibres  endowed  with  par- 
ticular  motor  properties,  the  altération  of  which  détermines  hemi- 
chorea. A  délicate  anatomo-pathological  analysis,  guided  by  clinical 
observation,  will  perhaps  some  day  succeed  in  circumscribing 
exactly  the  adjacent  régions  corresponding  to  thèse  two  orders  of 
fascicles. 

Along  with  post-hemiplegic  hemichorea  we  may  mention  a  patho- 
logical  condition  which  is,  as  it  were,  the  converse  of  this,  that  is, 
a  condition  in  which  the  choreiform  movements,  suddenly  developed 
in  the  limbs  of  one  side  of  the  body,  after  the  apoplectic  shock, 
soon  give  place  to  more  or  less  complète  hemiplegia.  Hemianses- 
thesia  usually  accompanies  this  kind  of  hemichorea,  which  may  be 
termed  prœ-hemiplegic.  Cases  of  this  kind  are,  no  doubt,  rather  rare  ; 
I  hâve  not  collected  more  than  three  examples.  An  autopsy  was 
made  in  ouly  one  of  them.  A  hœmorrhagic  focus  was  found,  about 
the  size  of  a  small  nut,  which  distended  the  posterior  half  of  the 
optic  thalamus.  The  patient  had  succumbed  about  three  weeks  after 
the  invasion  of  the  apoplectic  symptoms.  A  complète  and  absolute 
hemiplegia  had  replaced  the  hemichorea,  three  days  after  the 
invasion. 

According  to  what  has  been  stated  above  it  is,  evidently,  not  by 


284  SYMPTOMATIC   HEMICHOEEA. 

disorganising  a  part  of  the  optic  thalamus  that  the  hgemorrhage  hère 
produced  either  the  heraichorea,  or  the  heiniansesthesia.  Hère  two 
orders  of  syrnptoms  ought^  apparently,  to  be  both  correlated  to  the 
effects  of  the  compression  which  had  borne  upon  the  internai  capsule 
and  the  foot  of  the  corona  radiata,  in  the  immédiate  neighbourhood 
of  the  focus. 

Hemichorea,  whether  accompanied  by  hemiansesthesia  or  not, 
may  again  be  produced,  not  now  suddenly,  but  in  a  slow  and  pro- 
gressive manner,  and  without  being  necessarily  either  followed 
or  preceded  by  liemiplegia,  in  conséquence  of  the  development  of 
certain  neoplasias  in  the  substance  of  the  hémisphère.  Cases  of 
this  kind  are  rather  frequently  met  with  ;  and  I  quoted,  on  a  former 
occasion,  several  remarkable  examples.  It  is  as  probable  as  can  be 
that  the  morbid  products  which  détermine  such  effects  dépend  on  a 
localisation  analogous  to  that  which  we  endeavoured  just  now  to 
détermine  in  connection  with  hsemorrhagic  foci  ;  but  we  do  not  yet 
possess  any  positive  data,  with  respect  to  them.  This  will  be  an 
interesting  subject  for  future  research.  The  patient,  whom  I  am 
now  about  to  introduce  to  your  notice,  evidently  belongs  to  the 
category  of  cases  which  I  hâve  just  pointed  out. 

Slie  is  about  60  years  of  âge.  She  has  been  suffering  for  some 
iifteen  years  with  wandering  pains,  occupying  the  whole  extent  of 
the  right  upper  limb.  From  1869,  she  became  subject  to  epilepti- 
form  crises,  rather  imperfectly  defined,  and  towards  the  same 
period  this  same  upper  extremity  was  seized  with  choreiform 
trembling.  The  tremor  in  question  is,  as  it  were,  permanent  ;  it 
becomes  manifestly  exaggerated  in  purposed  movements,  but  it 
subsists  irrespective  of  any  voluntary  act.  It  is,  besides,  brought 
much  doser,  by  the  gênerai  aspect  of  its  characters,  to  the  shaking 
of  chorea  than  to  the  trembling  proper  to  paralysis  agitans  or  senile 
"  palsy.'^  I  will  add  that  a  total  hemiansesthesia,  with  participation 
of  the  spécial  sensés,  has  been  observed  in  this  patient,  during  the 
whole  of  last  year.  It  occupied  the  right  side  of  the  body;  at 
the  présent  moment,  spécial  sensibility  appears  to  hâve  been  re- 
established  throughout,  and  as  to  gênerai  sensibility  it  has  again 
become  nearly  normal  in  the  face,  the  trunk,  and  the  lower  extre- 
mity of  the  right  side.  The  right  upper  limb  alone,  the  seat  of 
choreiform  movements,  still  présents  throughout  its  whole  extent  a 
well-marked  weakening  of  tactual  sensibility. 

In  concluding,  I  would,  yet  once  more,  bring  out  tlie,  at  least. 


CHOREA  MINOE.  285 

external,  analogies,  which  draw  together  the  symptomatic  clioreas, 
connected  with  coarse  lésion  of  the  encephalon,  and  common  chorea. 
The  lattei-j  like  the  former,  may  remain  temporarily  at  least  con- 
fined  to  one  side  of  the  body  ;  it  is  often  accompanied  by  hemian- 
œsthesia,  it  may  be  preceded  or  followed  by  hemiplegia,  &c.,  &c.  ; 
in  short,  the  différence  that  séparâtes  thèse  two  orders  of  affec- 
tions, which  are  so  radically  distinct,  from  a  nosographic  point  of 
view,  is  rather  perhaps  in  what  we  are  accustomed  to  call  the  nature 
of  the  disease  than  in  its  anatomical  position.  If  the  latter,  so  far 
as  symptomatic  choreas  are  concerned,  were  once  exactly  determined, 
we  would  know  at  least  one  of  the  régions  of  the  encephalon  where 
we  should  seek  for  those  délicate  altérations  to  which  the  symptoms 
of  common  chorea  are  due. 


LECTURE  XX. 

ON  PARTIAL  EPILEPSY  OF   SYPHILMIC  ORIGIN. 

SuMMARY. — Partial  or  hémiplégie  ejpilepsy.  Ils  relations  witli 
cerehral  syphilis.  Historical  considérations.  Description  of 
a  case  of  partial  epilepsy  of  syphilitic  origin.  Characters  and 
peculiar  seat  of  the  cephalalgia.  Necessity  of  energetic  thera- 
peutical  intervention. 

Modes  of  invasion  of  the  convulsive  symptoms.  Neio  cor- 
roborative  examples.  Succession  of  fits.  Appearance  of  per- 
manent contractures.  Relations  hetioeen  the  cephalalgia  and 
the  motor  région  qf  the  hrain. 

Lésions.  Gummatous  pachymeningitis.  Probahle  seat  of 
those  lésions. 

Mixed  treatment,  with  interruptions. 

GENTLEMEN,^P«?'^iâ'^  01  hémiplégie  epilepsy,  of  which  I  recently 
endeavoured  to  make  you  grasp  the  principal  symptomatic  cha- 
racters and  varieties,  basing  my  remarks  on  the  description  of 
Bravais/  on  the  more  récent  work  of  Dr.  H.  Jackson,  of  London, 
and  also  on  my  own  observations,  is  one  of  the  most  fréquent 
manifestations  of  cerehral  syphilis.  That,  we  may  say,  is  a  fact 
emphatically  recognised  and  proclaimed  to-day,  amongst  our  British 
brethren,  as  witness,  among  others,  the  writings  of  R.  B.  Todd, 
those  of  MM.  Jackson,  Broadbent,  T.  Buzzard,^  and  some  others. 
On  the  other  hand,  in  France,  if  I  mistake  not,  it  has  not  yet  been 
remarked  as  much  as  it  deserves  to  be  on  account  of  its  practical 
interest,  although  one  of  the  most  compétent  authorities  on  thèse 
niatters,  Dr.  A.  Eournier,  laboured  last  year  to  popularise  a  know- 

1  'Thèse  de  Paris,'  No.  ii8,  t.  iv,  1827. 

^  Broadbent:    'The  Lancet/  21   Feb,,   1874.     T.  Buzzard  :    '  Aspects  of 
Syphilitic  Nervous  Affections,'  London,  1874. 


CLINIOAL    CASE.  287 

ledge  of  it,  in  a  work  which  I  caiinot  too  strongly  recommend  to 
your  considération.^  Consequently  you  will,  I  trust,  regard  it  as 
opportune  if  I  call  your  attention,  for  a  moment,  to  a  subject  which 
has  yet  been  perhaps  insuflBciently  studied,  and  give  you  a  summary 
abstract  of  a  certain  number  of  rather  regular  examples  of  partial 
epilepsy  of  syphilitic  origin,  which  I  hâve  lately  had  occasion  to 
observe.  In  the  course  of  my  statement  I  will  try,  as  we  proceed, 
to  give  you  an  opportunity  of  laying  your  finger  on  certain  pecu- 
liarities  which  are  often  exhibited  by  this  clinical  form  of  cérébral 
syphilis.  But,  I  shall  always  hâve  at  heart  to  render  prominent  the 
fact  that,  in  such  cases,  the  opportune  administration  of  appropriate 
agents,  when  it  is  resolutely,  I  was  about  to  say,  daringly  carried 
out,  according  to  a  certain  method,  may  triumph,  and  sometimes 
very  rapidly  triumph  over  ail  obstacles,  and  bring  about  a  lasting 
cure  even  in  cases  where  the  same  agents,  administered  on  other 
principles,  or  at  least  more  timidly,  would  hâve  completely  failed. 

I. 

On  the  i3th  December,  1874,  I  was  called  by  Dr.  Malhéné  to 
see  M.  X — ,  aged  40,  affected  by  serions  cérébral  symptoms,  and 
conflned,  on  this  account,  to  his  room  for  several  months.  In  his 
statement,  M.  X — ,  carries  the  présent  disease  back  to  the  month 
of  July,  of  the  same  year.  Having  been  employed  in  a  banking 
house,  lie  was  seated,  one  day  as  usual,  at  his  desk  engaged  in 
writing,  when,  suddenly,  without  having  noticed  any  immédiate 
precursory  phenomena  he  felt,  not  without  dismay,  his  right  lower 
limb  shaken  by  convulsive,  rhythmical,  hurried,  and  very  energetic 
jerks.  This  species  of  trépidation  lasted  for,  perhaps,  some  seconds, 
then  the  rigid  lower  extremity  rose  in  one  pièce,  and  immediately 
afterwards  M.  X —  fell  to  the  ground,  senseless.  He  did  not 
recover  consciousness  for  about  an  hour,  and  he  knows  nothing  of 
what  happened  during  that  time.  On  the  morrow,  he  was  able  to 
return  to  business,  and  no  new  accident  had  been  experienced, 
when,  one  day  in  September,  just  as  he  was  getting  down  from  au 
omnibus  he  fell  on  the  pavement,  in  a  state  of  unconsciousness, 
after  having  experienced  for  some  seconds,  as  before,  the  selfsame 
trépidation  with  rigidity  of  the  right  lower  extremity,  already  men- 
tioned.     A  slight  paretic  weakening  of  the  limbs  of  the  right  side, 

1  "De  l'épilepsie  syphilitique  tertiaire,"  leçon  professée,  par  A.  Pournier, 
(Clinique  de  Lourcine.)     Paris,  1876. 


'ioo  DESCRIPTION    OF  FIT. 

a  noticeable  confusion  of  ideas,  a  certain  degree  of  dulness  of  the 
nfind,  sucli  were  the  symptoms  whicli  followed  this  second  attack, 
and  which  hâve  persisted  after  it.  From  this  period,  M.  X —  gave 
up  work,  and  left  his  dwelling  only  at  rare  intervais,  chiefly  because 
he  was  always  in  dread  of  being  seized  in  the  street,  with  another 
attack. 

Towards  the  middle  of  the  month  of  November,  without  appa- 
rent cause,  without  any  warning,  the  third  attack  smote  him  ;  this 
time  the  duration  of  the  phenomena  of  the  motor  mira  continued 
longer,  and  the  patient,  before  losing  his  sensés,  had  time  to 
perceive  that  the  convulsive  rhythmical  shaking,  as  well  as  the 
rigidity,  after  occupying  the  right  lower  limb,  did,  without  aban- 
doning  this  position,  rapidly  invade  the  upper  limb  on  the  same 
side.  A  spectator  of  the  accident  relates  that  the  head  was  next 
twisted  towards  the  right  shoulder,  whilst  the  right  side  of  the 
face  was  grimacing  ;  then  the  convulsions  extended  to  the  whole 
body,  but  still  predominated  on  the  left  side;  and  after  their 
cessation,  stertorous  sleep  supervened.  It  is  certain  that  during 
the  fit  M.  X —  did  not  bite  his  tongue,  nor  micturate  under  him. 
Whilst  still  in  a  state  of  unconsciousness  several  other  fits  occurred, 
ail  similar  in  every  respect  to  the  first,  so  as  to  constitute  a  status 
which  lasted  for  about  three  hours.  The  consécutive  phenomena, 
mentioned  already  in  connection  with  the  September  fit,  simply 
became  more  marked  after  the  last-mentioned  attack  :  for  some 
hours  there  was  found,  in  addition,  a  certain  degree  of  difficulty 
in  speaking  and  verbal  amnesia,  a  feeling  of  numbness  in  the 
cheek  of  the  right  side,  in  the  vicinity  of  the  labial  commissure  ; 
but  the  latter  symptoms  hâve  been  quite  transitory,  they  had  quite 
disappeared  when  I  saw  M.  X — . 

After  verifying  the  existence  of  the  weakening  of  the  right 
extremities,  of  which  I  had  been  informed,  and  which  was  only 
slight,  I  remarked  that  they  were  not  the  seat  of  any  feeling  of 
formication,  and  that  they  did  not  show  any  trace  of  anœsthesia. 
Lastly,  I  found  that  the  vision  was  nowise  disturbed. 

On  hearing  the  narrative  of  M.  X — ,  I  had  been  naturally  led 
to  suspect  that,  in  his  case,  syphilis  might  be  at  work,  and  I  at 
once  proceeded  to  an  examination  of  the  difi'erent  parts  of  he  body 
accessible  to  the  eye,  hoping  to  meet  the  traces  of  some  one  of  the 
later  manifestations  of  that  disease.  ïhe  resuit  of  this  investigation 
was  wholly  négative. 


CHAEACTERS  OF  CEPHALALGIA.  289 

It  was  not  so  as  regards  tlie  study  of  his  antécédents,  which,  on 
the  contrary,  enabled  me  to  gather  some  very  significant  informa- 
tion. I  learned,  in  fact,  what  follows  : — At  the  âge  of  twenty-nine, 
that  is  to  say,  twelve  years  before  the  appearance  of  the  first 
epileptiform  attack,  M.  X —  had  contracted  a  hard  chancre,  soon 
fo]lowed  by  varions  manifestations  due  to  constitutional  syphilis, 
roseola  amongst  the  rest.  The  treatment  of  the  disease,  at  this  period, 
seems  to  havebeen  regularly  directed  andcontinued  forseveralmonths- 

Matters  remained  so,  and  for  more  than  ten  years  M.  X —  had 
lived  in  good  health,  enjoying  perfect  security,  when,  towards  the 
close  of  1873,  he  began  to  expérience  a  singular  indisposition, 
chiefiy  denoted  by  great  prostration  of  strength,  inaptitude  for 
intellectual  work,  well-marked  dyspeptic  disturbances,  which  were 
very  tenacious  and  showed  theraselves  rebellions  to  the  administra- 
tion of  the  usual  remédies.  A  certain  degree  of  wasting,  a  rather 
marked  cachectic  state,  which  no  viscéral  affection  seemed  to 
account  for,  and,  lastly,  a  cephalalgia  of  a  peculiar  kind  arrived 
soon  after  to  fiU  up  the  picture. 

This  cephalalgia  has  never  entirely  ceased  to  exist  to  a  certain 
extent  since  then.  I  hâve  not,  however,  discussed  it  hitherto, 
whilst  enumerating  the  symptoms,  because  I  desired  to  commend 
it  specially  to  your  attention.  At  the  outset,  it  was  always  localised 
in  a  circumscribed  space,  not  larger  than  a  franc,  above  the  right 
eyebrow,  towards  the  temple.  Afterwards,  when  an  exacerbation 
occurred,  it  often  extended  to  the  top  of  the  head,  and  even  to  the 
occiput,  without,  however,  ever  quitting  its  primary  position.  It 
appears  to  be  well  established  that  the  exacerbations  take  place, 
usually,  towards  seven  o'clock  in  the  evening,  extending  more  or 
less  into  the  night,  and  sometimes  hindering  sleep  :  they  hâve  never 
been  followed  by  vomiting. 

If  I  insist  upon  this  head-pain,  it  is  because  you  will  find  the 
same  phenomenon,  with  the  pecuharities  just  described,  in  the  history 
of  many  cases  of  syphilitic  epilepsy.  This  fact  has,  indeed,  been 
several  times  pointed  ont  by  the  authors  who  hâve  occupied  them- 
se-lves  with  thèse  questions  :  "  If  pain  in  the  head,"  remarks,  for 
instance,  Dr.  Buzzard,  "be  associnted  with  the  convulsive  attacks, 
it  generally  précèdes  the  outbreak  in  syphilitic  convulsion,  and  is 
often  localised  in  one  particular  spot.  There  is  frequently  a  history 
of  antécédent  pain  for  months  before  the  first  fit."  ^  Unquestionably 
^  T.  Buzzard,  loc.  cit.,  p.  14. 

VOL.  II.  19 


290  TEEATMENT. 

we  ought  notj  by  any  means,  to  go  so  far  as  to  compare  this 
ceplialalgia^  fixed  in  one  spot,  and  long  preceding  the  convulsive 
attack  as  a  characteristic  sign  ;  it  may,  in  truth,  be  met  with  in 
différent  forms  of  partial  epilepsy,  independent  of  syphilis.  Never- 
theless,  in  this  disease,  it  is  generally  much  more  marked  than 
anywhere  else,  hence  it  is  an  élément  which  the  clinical  observer 
should  not  disdain  to  utilise,  since  it  may  sometimes  coutribute  to 
elucidate  the  diagnosis. 

After  collecting  the  information  which  bas  been  related,  I  believed 
myself  authorised  to  déclare  that  the  différent  accidents,  experienced 
by  M.  X — ,  for  eighteen  months,  were  attributable  to  syphilis  and 
that  probably  they  would  give  way  to  the  properly  direoted  use  of  the 
mixed  treatment.  I  was  then  informed  that,  in  accordance  with  the 
advice  of  a  physician  who  had  been  previously  consulted,  M.  X — , 
for  more  than  a  year,  had  perhaps  never  completely  ceased  to 
make  use  of  either  syrup  of  iodide  of  mercury,  or  of  iodide  of 
potassium,  takeu  in  médium  doses.  This  révélation  did  not  dis- 
courage me,  and,  relying  on  the  expérience  of  former  cases,  I 
expressed  the  opinion  that  we  should  hère  proceed,  as  it  were,  by 
z.  pJiysical-force  assault  and  endeavour  to  precipitate  the  dénouement; 
that,  in  other  words,  the  immédiate  administration  of  large  doses 
would  triumph  perhaps,  rapidly,  where  the  prolonged  action  of 
médium  doses  had  been  found  inefficient  to  prevent  the  occurrence 
of  the  symptoms  and  to  combat  them,  when  once  developed.  We 
agreed,  Dr.  Malhéne  and  myself,  to  prescribe  a  treatment  as  fol- 
lows  :  each  day,  5  or  6  grammes  of  Neapolitan  unguent  was  to  be 
rubbed  in,  whilst,  at  the  same  time,  iodide  of  potassium  should  be 
taken  in  doses  of  from  6  to  8  or  i  o  grammes  in  the  twenty-four 
hours,  one  portion  to  be  swallowed,  the  other  to  be  injected  as  an 
enema.  The  treatment  should  be  continued,  as  much  as  possible, 
in  ail  its  rigour  for  about  twenty  days,  then  completely  suspended  for 
some  days  ;  recommenced  anew  in  the  same  manner  as  at  first, 
and  so  on  for  three  or  four  turns. 

I  saw  M.  X — ,  at  the  end  of  1875.  He  informed  m«  that  the 
treatment  had  been  begun  on  the  day  following  the  consultation  ; 
that,  two  months  later,  the  improvement  in  ail  the  permanent 
symptoms — cephalalgia,  paresis,  dyspepsia,  cachectic  condition — 
was  such  that  he  had  already  been  able  to  résume  his  business  ; 
that,  a  month  later,  he  regarded  himself  as  completely  cured  ;  that, 
lastly,  as  to  the  epileptiform  attacks,  they  had  not  again  reappeared, 


ANOTHEE    CASE    OF    SYPHILITIO    PARTIAL    EPILEPSr.    291 

and  that  he  had  not  experienced  any  sensation  which  would  make 
him  appreheud  their  return. 

I  saw  M.  X — ,  again,  at  the  end  of  1876.  At  this  period,  his 
recovery  had  never  been  marred  for  a  single  instant. 

IL 

As  I  hâve  already  had  occasion,  several  times,  to  point  ont  to 
you,  it  is  in  one  of  the  upper  extremities,  or,  in  one  side  of  the 
face,  that  the  outbreak  of  the  convulsive  attacks  occur,  in  the  great 
majority  of  cases  of  partial  epilepsy,  whatever  its  origin  œay  be.^ 
Hence,  an  invasion  which  should  first  begin  by  assaihng  one  of  the 
lower  extreraities  ought  to  be  considered,  in  this  category,  as  a 
rare  and  exceptional  fact.  Yet,  we  hâve  just  seen  this  mode  of 
invasion  noted  in  the  preceding  observation  ;  owing  to  a  curions  con- 
currence of  circumstances,  we  are  about  to  meet  with  it,  once 
again,  in  the  following  case,  in  which  as  in  the  former,  cérébral 
syphilis  was  the  disease  at  work. 

A  foreign  physician,  passing  through  Paris,  requested  me, 
August  26,  187-,  to  visit  him  in  order  to  advise  him  in  the  follow- 
ing circumstances: — When  dining  with  a  friend,  on  the  second 
previous  evening,  he  had  been  tormented,  during  the  entire  time  of 
the  dinner,  by  the  exaspération  of  a  headache  from  which  he  had 
been  suffering,  in  a  moderate  degree,  for  some  days.  On  rising  from 
table,  he  resolved  to  return  home  immediately,  on  foot  ;  but  he  had 
only  taken  a  few  steps  in  the  street  when,  suddenly,  his  right  lower 
extremity  became  rigid,  and,  at  the  same  time,  was  shaken,  in  some 
sort,  by  hurried  and  violent  rhythmical  convulsions.  Almost  imme- 
diately after  the  upper  limb,  of  the  same  side,  was  invaded  in  its 
turn,  and,  at  this  moment  M.  B —  fell,  insensible,  on  the  pathway. 
On  recovering  consciousness  he  was  much  surprised  to  find  himself 
reposing  in  his  own  bed,  whither  he  had  been  carried.  The  time 
during  which  he  was  insensible  may  hâve  lasted  for  an  hour. 

During  the  night  he  was  sleepless,  and  during  the  following  day 
attacks  of  the  same  kind  came  on  two  or  three  times.  None  of 
them,  however,  went,  like  the  first,  so  far  as  loss  of  consciousness. 
Each  time  they  occurred  the  patient  assisted,  not  unmoved,  at  the 

1  This  fact,  already  pointed  out  by  Bravais,  has  been  still  more  explicitly 
described  by  Dr.  H.  Jackson,  "  A  Study  on  Convulsions,"  in  '  Transactions  of 
the  St.  Andrew's  Médical  Graduâtes'  Association,'  t.  iii,  1870.  My  own  ob- 
servations fuUy  coufirm  it. 


292  CEKEBEAL   SYPHILIS. 

graduai  and  regular  invasion  of  the  convulsive  movements  which, 
alv^ays  beginuing  by  the  left  lower  limb,  next  took  possession  of  the 
îipper  extremity  on  the  same  side,  and  sometimes  also  of  the  cor- 
responding  half  of  the  face.  A  new  attack,  which  was  also  an 
abortive  one  like  those  of  the  day  before,  had  taken  place  in  the 
moming  of  the  day  in  which  I  saw  M.  B —  for  the  first  time. 
Diiring  ail  this  period  the  headache  had  not  ceased  to  plague  him, 
becoming  cruelly  exasperated  at  the  instant  when  the  convulsive 
phenomena  were  about  to  show  themselves. 

I  found  M.  B —  to  be  an  individual  in  the  prime  of  manhood, 
tall,  strongly  made,  and  habitually  enjoying  excellent  health  ;  for 
some  weeks,  however,  he  had  been  feeling  indisposed,  without  appe- 
tite,  heavy,  easily  fatigued  by  the  least  effort,  and,  moreover,  his 
features  had  grown  visibly  paler.^  Having  noted,  at  the  outset,  in 
this  case,  the  absence  of  motor  paralysis,  and — headache  excepted — 
of  ail  disturbance  of  sensibility,  alike  in  face  and  in  limbs,  I  readily 
perceived  that  there  existed  a  certain  degree  of  confusion  in  his 
ideas,  and  perhaps  also  a  little  trouble  in  speaking,  without,  however, 
any  symptoms  of  aphasia. 

The  question  of  remote  antécédents  was  particularly  interesting. 
M.  B —  confided  to  me  that,  eighteen  months  before,  he  had  contracted 
a  hard  chancre,  and  that,  subsequently,  various  diathetic  symptoms 
had  appeared,  amongst  the  number  being  palmar  psoriasis,  traces  of 
which,  indeed,  could  still  be  remarked. 

I  did  not  hesitate,  as  you  may  guess,  to  connect  the  nervous  dis- 
turbances  which  M.  B —  experienced  with  the  syphilis  from  which 
he  had  suffered,  and  I  advised  him  to  act,  consequently,  in  a  prompt 
and  energetic  manner.  It  was  agreed  that  the  treatment  should  be 
begun  in  accordance  with  the  plan  described  when  speaking  of  the 
case  of  M.  X — ,  and  put  in  practice  immediately.  The  administration 
of  iodide  of  potassium  and  mercurial  frictions  were  commenced  the 
same  day.  An  abortive  attack,  this  time  confined  to  the  lower  extremity, 
took  place  once  more  on  the  morrow,  or  after  morrow  :  it  was  the 
last,  and  at  the  end  of  a  fortnight  his  gênerai  health  had  become  so 
much  improved  that  M.  B —  was  able  to  return  to  his  native  land. 

During  a  short  stay  which  I  made  in ,  about  a  year  after  the 

occurrence  of  the  24th  August,  187-,  I  had  the  pleasure  of  meeting 

1  With  respect  to  tlie  cachectic  state  and  eartliy  pallor  which  individuals, 
affected  with  cérébral  syphilis  habitually  présent,  see  the  interesting  remarks  of 
Mr.  Buzzard,  in  référence  to  diagnosis,  loc.  cit.,  p.  'è'^. 


THIED   CASE   OF    STPHILITIO    EPILEPSY.  293 

M.  B —  at ,  and  of  finding  him  in  a  state  of  perfect  health.  The 

treatment,  prescribed  in  Paris,  had  been  followed,  with  due  inter- 
ruptions, for  the  space  of  about  three  months.  No  nervous 
disorder  had  reappeared. 

It  may  happen  that  attacks  of  syphilitic  partial  epilepsy  are  pre- 
ceded  by  a  certain  number  of  fits,  in  which  loss  of  consciousness 
suddenly  occurs,  quite  unexpectedly,  without  immédiate  forewarning 
symptoms,  whilst  the  convulsive  movements  simultaneously  show 
themselves  everywhere;  and  thus,  consequently,  we  get  the  classic 
picture  of  common  epilepsy.  The  case,  of  which  I  am  about  to 
state  the  principal  détails,  gives  us  an  example  of  this  kind.  It  also 
présents  a  certain  number  of  other  interesting  peculiarities. 

M.  K — ,  born  in  the  Antilles,  of  a  délicate  constitution,  and  in  the 
highest  degree  impressionable,  was  attacked  with  indurated  chancre 
in  1868,  at  the  âge  of  39.  Among  the  syphilitic  manifestations, 
which,  sooner  or  later,  followed  on  the  primary  disease,  was  a  double 
iritis  of  extrême  tenacity,  spots  (?)  on  the  brow,  palmar  psoriasis,  in- 
tense and  prolonged  rheumatic  pains,  great  and  lasting  ansemia,  and, 
finally,  subacute  arthritis  which  had  chiefly  affected  the  tibio-  tarsal 
articulations.  Treatment,  suitable  to  the  circumstances,  had  been 
followed  almost  continuously  for  about  six  months  and  was  then 
finally  abandoned. 

Ail  went  well  till  1873.  Towards  the  close  of  this  year,  M.  K — , 
who,  for  several  months,  had  fiUed  an  officiai  post  in  Cochin-China, 
was  attacked  with  the  diarrhœa  of  the  country,  and,  owing  to  this, 
he  became  extremely  weak.  Towards  the  same  period,  he  began  very 
often,  almost  constantly,  to  suffer  from  pains  in  the  head,  which  he 
believed  to  be  megrims,  and  which  persisted,  growing  worse,  up  to 
récent  times. 

In  May,  1874,  about  six  years  after  the  outbreak  of  syphilis, 
although  the  diarrhœa  had  a  little  improved  for  some  time,  M.  K — , 
who  was  always  very  weak,  and  always  suffering  from  headaches, 
was,  after  an  excited  discussion,  suddenly  attacked  with  an  epileptic 
fit,  with  immédiate  loss  of  consciousness,  convulsions  generalised 
from  the  outset,  foaming  at  the  mouth,  and  involuntary  micturition, 
&c.  The  invasion,  I  repeat,  appears  to  hâve  been  literally  sudden, 
unannounced,  and  the  patient  knows  nothing  of  the  fit,  save  what 
he  was  afterwards  told  by  those  présent. 

In  conséquence  of  this  attack  it  was  decided  that  M.  K — ,  on 
acBount  of  the  long  ailing  state  of  his  health,  should  take  unlimited 


294  DESCEIPTION    OF  FITS. 

leave  and  return  to  France.  On  the  steamboat,  during  the  voyage 
home,  a  new  fit  came  on,  quite  similar  to  the  first  ;  theii,  some  days 
after,  another,  but  this  one  was  very  différent  from  the  previous 
attaclcs.  On  this  occasion,  the  patient  had  first  felt  bis  left  hand 
close  up  convulsively  and  the  arm  stiffen,  then  the  neck  twisting 
round  brought  bis  face  towards  the  left  shoulder.  Lastly,  as  tbough 
drawn  by  an  invincible  force  towards  the  left,  he  fell  over  on  that 
side,  and  it  was  at  this  moment  only,  that  is,  some  seconds  after  the 
invasion  of  the  attack,  that  loss  of  consciousness  supervened.  The 
true  character  of  the  convulsive  symptoms  was  thus  revealed, 
henceforth,  the  fits  never  again  made  their  reappearance  but  under 
the  form  of  partial  or  hémiplégie  epilepsy,  sometimes  with,  oftener 
probably  without,  loss  of  consciousness. 

From  the  9th  July,  when  the  patient  landed  at  Marseilles,  to  the 
end  of  October,  in  other  words,  during  a  period  of  neariy  four 
months,  they  hâve  never  ceased  to  appear  every  five  or  six  days, 
and  sometimes  even  several  times  a  day. 

During  the  three  first  months,  if  we  except  the  cephalalgia  which 
was  almost  always  présent  and  localised  as  I  shall  describe,  in  a 
circumscribed  spot  in  the  right  pariétal  région,  the  intervais  between 
the  attacks  remained  free  from  any  persistent  symptom  ;  but,  in  the 
first  days  of  October,  the  contracture  began  to  take  possession  of 
the  left  upper  limb,  of  the  hand  especially,  and  to  occupy  it  in  a 
persistent  manner  so  as  to  keep  it,  permanently,  in  semiflexion. 
It  even  invaded,  though  to  a  less  extent,  the  corresponding  lower 
extremity.  It  should  be  added  that  the  contractured  hand  and 
forearm  had,  at  the  same  time,  become  the  seat  of  disagreeable  for- 
mication,  and  also  of  an  exquisite  hyperresthesia;  the  patient  dreaded 
extremely  lest  this  limb  should  be  struck,  or  even  lightly  touched  ; 
and  if  this  happened,  by  chance,  to  occur,  he  shouted  with  the 
pain.  He  declared  that,  on  several  occasions,  a  shake  experienced  by 
his  painful  hand  had  given  rise  to  one  of  his  convulsive  attacks.' 

It  will,  probably,  not  be  out  of  place  to  give  you  a  description  of 
the  principal  phenomena  which  mark  thèse  attacks,  such  as  I  hâve 
received  them  from  the  lips  of  a  very  intelligent  person,  who  wit- 
nessed  the  principal  phases  of  M.  K — 's  disease.  I  shall  also 
utilise,  in  my  statement,  the  observations  made  by  the  patient 

^  It  is  not  unkuown  that  fits  of  partial  epilepsy  of  cérébral  origiu  may 
be  provoked  by  certain  acts.  In  one  of  my  patients,  named  P — ,  affected 
by  contracture  of  the  upper  and  lower  limbs  of  the  left  side,  which  is   to 


DESCRIPTION    OF  FITS.  295 

liimself  during  the  occurrence  of  those  of  his  attacks  in  which 
consciousness  was  preserved, 

The  invasion  of  the  convulsions  is  constantly  announced  by 
an  exaspération  of  the  cephalalgia  localised,  as  has  been 
said,  in  a  spot  of  the  right  pariétal  région.  The  pain,  at 
this  moment  takes  a  throbbing  character,  and,  at  the  end  of 
some  minutes,  it  seems  to  spread  over  half  the  face  and  neck  of 
the  same  side.  Warned  by  thèse  premonitory  phenomena,  the 
patient  has  almost  always  time  to  get  to  bed,  and  lie  down.  Then, 
the  left  upper  limb  is  seen  to  become  excessively  flexed,  at  the  wrist- 
and  elbow-j oints,  and  at  the  same  time  to  assume  the  attitude  of 
forced  pronation  ;  some  seconds  after,  rhythmical  shocks  are  experi- 
enced  which  shake  it  throughout  its  whole  length.  The  h  and  is 
soon  brought  round  towards  the  left  shoulder,  and  is  also  shaken 
by  the  same  jerks  ;  at  the  same  time,  on  the  left  side  of  the  face, 
grimaces  occur  and  are  rapidly  repeated.  The  left  lower  extremity 
is  invaded,  in  its  turn,  it  stiffens  out  in  forced  extension,  rises 
above  the  level  of  the  bed,  then  after  some  seconds  it  is  taken  with 
trépidation.  Lastly,  in  certain  fits,  the  stifFness  and  rhythmical 
convulsions  invade  the  régions  of  the  opposite  side  of  the  body. 
When  loss  of  consciousness  takes  place,  this  is  the  moment  at 
which  it  supervenes.  I  should  add  that  the  patient,  after  having 
experienced  the  feeling  of  being  drawn  towards  the  left,  several 
times  became  subject,  during  the  fit,  to  a  real  rotation-movement, 
from  right  to  left,  round  the  longitudinal  axis  of  the  body,  and,  at 
the  end  of  the  attack,  has  found  himself  lying  prone,  his  face  to 
the  ground.^ 

some  extent  permanent,  but  which  becomes  considerably  exasperated 
when  she  stands  or  walks,  the  spontaneous  fits  begin,  by  the  lower  extre- 
mity, and  the  latter  then  becomes  excessively  stiifened  in  extension,  the 
foot  taking  the  attitude  of  spasmodic  talipes  equinus,  and  trépidation  soon 
supervenes.  The  upper  limbs,  and  then  the  face,  are  next  invaded,  succes- 
sively,  and  loss  of  consciousness  supervenes  in  certain  cases.  When  tbe  fits 
hâve  not  appeared  for  some  time  it  is  always  possible  to  provoke  artificially 
their  development  by  suddenly  turning  up  the  top  of  the  left  foot  ;  trépidation 
almost  certainly  shows  itself  as  a  conseqnence,  and  ail  the  other  phenomena 
of  the  fits  foUow. 

1  It  is  remarkable  that,  in  the  case  of  M.  K — ,  the  attacks  came  on,  nearly 
always  between  five  and  six  o'clock  in  the  evening.  M.  Lagneau  fils,  '  Maladies 
syphilitiques  du  système  nerveux,'  Paris,  1860,  p,  125,  has  coUected  several 
examples  of  syphilitic  epilepsy,  in  which  the  fits  came  on  chiefly  in  the  eveuing 
or  night. 


296  LOCALISATION  OP  THE    CEPHALALGIA. 

Sucli  is  tlie  regular  and  constant  order  of  the  succession  of  con- 
vulsive  phenomena.  I  believe  I  ought  to  mention  to  you,  inci- 
dentally,  that  the  mode  of  invasion  takes  place  hère  in  conformity 
with  therule  established  by  the  ingénions  studies  of  Dr.  H.  Jackson. 
You  hâve  not_,  in  fact,  forgotten  that,  according  to  this  distin- 
guished  physician — whose  assertions  with  respect  to  this  matter  I 
hâve  more  than  once  been  able  to  verify — when  convulsions  in 
partial  epilepsy,  commencing  by  the  upper  extremity,  tend  to  become 
generalised,  they  only  invade  the  lower  limb  after  having  first 
affected  the  face.  If,  on  the  contrary,  the  case  be  one  in  which  the 
face  is  first  affected,  the  upper  extremity  is  the  next  taken  ;  and, 
lastly,  cornes  the  turn  of  the  lower  limb.  Pinally,  if,  as  in  the  two 
first  observations  which  I  hâve  reported,  the  convulsions  should  first 
invade  the  lower  extremity,  they  spread  successively  to  the  upper 
limb  first,  and  then  to  the  face.  This  order  seems  to  be  almost 
never  inverted,  a  fact  which  is  not  merely  curious,  but  one  well 
adapted,  you  will  understand,  to  throw  light  upon  varions  ques- 
tions belonging  to  the  domain  of  pathological  physiology. 

IVom  this  same  point  of  view  of  physiological  interprétation,  I 
will  notice  that  the  cephalalgia,  the  exaspération  of  which  announced 
in  M.  K — ^s  case  the  development  of  the  fit,  occupied  a  circumscribed 
spot  in  the  right  pariétal  région,  whilst  the  convulsions  affected  parts 
on  the  left  side.  This  alternate  arrangement  of  the  convulsions  and 
the  head-pain  has  been  more  or  less  mentioned  in  a  certain  number 
of  cases  of  partial  epilepsy  of  syphilitic  origin,^  or  independently 
of  syphilis  ;  and  perhaps  the  relationship  in  question  may  be,  in 
similar  cases,  more  frequently  noted  in  future,  when  greater 
attention  shall  hâve  been  paid  to  seeking  it  out.  However  it  be, 
the  fact  is  one  worthy  of  interest  when  it  is  known  that  those  part» 
of  the  surface  of  the  cérébral  hémisphères,  which  are  in  relation 
with  the  pariétal  région  of  the  head,  and  more  particularly  the 
convolutions  which  border  on  Eolando's  fissure  (pariétal  and 
ascending  convolutions),  are  designated  by  récent  studies  as  repre- 
senting  the  motor  zone  ;  or,  in  other  terms,  as  the  only  région  of  the 
cérébral  cortex  whose  irritation  may  détermine,  on  the  opposite 
side  of  the  body,  the  production  of  the  phenomena  of  partial 
epilepsy.     You  should  not,  however,  expect  always  to   meet  an 

1  See,  araongst  others,  the  observations  of  Dr.  Tbdd  :  '  Clinical  Lecture  on 
Paralysis,'  &c.,  London,  1856.  Lecture  XVII,  "On  a  Case  of  Syphilitic 
Disease  of  the  Dura  Mater,"  p.  301. 


PKOGRESS  OF  THE    DISEASE.  297 

arrangement  of  convulsions  and  cephalalgia  so  exactly  in  conformity 
with  the  theory.  You  hâve,  in  fact,  seen^  in  our  first  observation, 
the  premonitory  head-pain  and  the  initial  convulsions  occupy  the 
same  side.  I  could  also  cite  some  other  examples  of  the  same 
kind.i 

But  it  is  time  to  return  to  the  particular  case  of  M.  K — .  During 
a  Icmg  period  of  four  months,  ill-directed  hydropathic  opérations, 
insignifîcant  doses  of  bromide  of  potassium  had  been  the  only 
agencies  opposed  to  the  progress  of  the  disease.  Thus,  the  position 
of  affairs  had  been  daily  growing  worse,  and,  towards  the  middle 
of  October,  it  had  become  most  disquieting.  The  attacks  came  on 
with  renewed  vigour,  amnesia,  hébétude,  a  genuine  dethronement  of 
the  intellect  supervened  ;  and  the  diarrhœa,  which  had  been  for  an 
instant  exorcised,  had  reappeared.  The  patientas  debility  had 
reached  its  climax.  Having  been,  for  several  weeks,  confined  to  his 
chamber,  he  had  now  found  it  completely  impossible  to  leave  his 
bed. 

Matters  had  reached  this  point,  when  Drs.  Cornuel,  Picard,  and 
myself,  met  in  consultation  on  M.  K — 's  case.  It  was  agreed  that 
as  energetic  action  as  the  gênerai  state  of  the  patient  allowed  should 
be  adopted.2 

He  was  put  upon  milk  diet,  and  nitrate  of  silver  was  prescribed 
in  the  form  of  pills  ;  at  the  same  time  mercurial  frictions  were  to 
be  employed,  and  iodide  of  potassium  administered  in  doses  of  from 
3  to  5  grammes  in  the  twenty-four  hours. 

Thanks  to  the  intelligent  co-operation  of  devoted  relatives,  our 
prescriptions  were  followed  to  the  letter.  The  results  obtained  were 
immediately  of  the  most  encouraging  character.  At  the  end  of 
only  eight  days  it  had  already  become  évident  that  the  disease  was 
not  beyond  the  resources  of  médical  art  ;  only  one  new  fit  had  taken 
place  on  the 3oth  October;  the  permanent  contracture  had  disappeared 
as  if  by  enchantment  ;  and  finally  the  patientas  gênerai  condition  had 

1  lu  several  cases  of  partial  epilepsy  which  I  hâve  observed,  the  premonitory 
head-pain  of  the  attacks  was  iocalised  in  two  spots  simuitaneously;  one  was 
situate  in  the  pariétal  région  on  one  side,  the  other  in  the  temporal  région  of 
the  opposite  side,  The  pariétal  pain,  in  thèse  cases,  always  occupied  the  side 
opposite  to  the  convulsions. 

2  The  good  eflfects  of  mercurial  préparations  against  the  cachectic  state, 
which  dépends  on  tertiary  syphilis  hâve  been  perfectly  illustrated  by  Dr.  Reade 
(of  Belfast),  in  an  interesting  passage  of  his  book,  '  Syphilitic  Affections  of 
the  Nervous  System,'  London,  1867,  p.  18. 


298  TEEATMENT. 

remarkablj  improved.  Eight  days  afterwards^  the  patient  was 
able  to  leave  liis  bed,  and  take  some  steps  about  his  room. 

At  the  beginniug  of  December,  he  had  become  able  to  leave  his 
dwelliiig,  and  to  take  rather  long  carriage  drives  ;  at  the  end  of  this 
monthj  he  had  several  times  walked  about,  in  the  open  air,  for  over 
an  hour  at  a  time. 

Unfortunately,  during  the  course  of  the  first  six  months  of  the 
year  1875,  the  diarrhœa,  which  he  had  contracted  in  Cochin-China, 
showed  itself  anew  at  différent  intervais  and,  in  conséquence  of  this, 
the  projected  répétition  of  the  mixed  intempted  treatment  could 
not  be  put  into  practice  in  a  regular  manner.  Three  or  four  times, 
during  this  period,  there  were  relapses  of  the  cérébral  affection, 
marked  by  epileptiform  attacks,  but  yet  less  intense  and  much  more 
rare  than  the  preceding  seizures.  But,  at  last,  during  a  sojourn  of 
several  months  at  Amélie-les-Bains,  the  state  of  the  intestines  having 
become  modified  in  the  happiest  manner,  the  treatment  of  the  con- 
vulsive  affection  could  be  taken  up  in  earnest,  and  continued  for  a 
sufficient  space  of  time.  At  the  end  of  this  treatment,  the  nervous 
symptoms  définit ely  disappeared. 

M.  K —  came  to  pay  me  a  visit  at  the  close  of  1876;  for  four- 
teen  months  he  had  not  experienced  any  further  attacks  ;  his  health, 
in  fact,  was  completely  restored.  He  was  about  to  leave  for  our 
American  colonies,  where  he  intended  to  résume  at  once  his  former 
ofiicial  functions. 

Undoubtedly,  it  would  only  be  self-deception  to  expect  always  to 
meet  with  such  fortunate  results  as  those  which  were  obtained  in  the 
three  preceding  observations,  and  I  am  not  ignorant  of  the  fact  that  it 
would  be  easy  to  quote  a  number  of  cases  of  cérébral  syphilis,  with 
partial  epilepsy,  where,  in  spite  of  the  assistance  given  by  an  en- 
lightened  zeal,  things  turned  ont  badly.  I  cannot  help  believing, 
however,  that,  in  cases  where  the  nature  of  the  nervous  symptoms 
is  recognised  in  time,  the  plan  of  treatment  proposed  might  be 
put  into  practice, — want  of  success  will  be  the  exception.^ 

'  Transient  motor  paralysis  of  a  limb,  supervenin^  suddeuly,  without  being 
preceded  by  contracture  or  touic  convulsions,  and  returning  repeatedly  after 
more  or  less  leugthy  iutervals,  ought  to  be  placed,  beside  partial  epilepsy, 
among  the  mauy  varions  manifestations  of  cérébral  syphilis. 

In  September  1872, 1  was  consultedby  M.  A — ,  cavalry  oflBcer,  for  a  cepha- 
lalgia  which  had  been  well-nigh  permanent  for  about  six  weeks,  and  which 
had  previously  siiown  itself  several  times  in  the  course  of  the  year.  In 
addition  to  the  head-pain,  there  were  well-marked  dyspeptic  disturbance. 


GUMMATOUS   PACHYMENINGITIS.  299 

The  lésions  of  circumscribed giunmatous pacliymemng'itis ,  witli par- 
ticipation of  the  subjacent  membranes,  appear  to  be  the  most  usual 

fréquent  vomitings,  great  prostration  of  strength,  emaciation,  and  extrême 
anEemia.  The  seat  of  the  cephalalgia  is  unfortunately  not  stated  in  the  note 
which  I  hâve  kept. 

M.  A — ,  had  contracted  a  hard  chancre  fifteen  years  before,  and  from 
that  until  a  récent  date,  he  had  not  ceased  to  be  subjected,  from  time  to 
time,  annually  so  to  speak,  to  mercurial  treatment,  and  chiefly  to  the  exhi- 
bition of  iodide  of  potassium,  with  the  object  of  combating  varions  symptoms 
which  were  always,  rightly  or  vrrongly,  referred  to  the  syphilis  by  the  phy- 
sicians  consulted.  Under  the  influence  of,  I  know  not  what,  préoccupations, 
I  misunderstood,  I  must  confess,  during  nearly  a  month,  the  true  character 
of  the  cephalalgia,  and  the  other  phenomena  which  accompanied  it.  Hence 
the  symptoms  grew  constantly  worse. 

One  day  I  was  informed  that,  for  some  time  past,  M.  A —  experienced  from 
time  to  time,  what  they  called  absences  (transient  loss  of  consciousuess).  He 
would  stop  suddeuly,  with  fixed  look,  in  the  middle  of  a  conversation,  grow 
pale,  and  at  the  close  of  one  of  those  lits,  which  barely  lasted  a  few  seconds, 
lie  would  remain  for  some  time  as  if  stupid.  Nothing  resembling  aphasia  was 
noticed,  nor  any  trace  of  convulsions  anywhere,  at  the  beginning  of  the  crisis, 
The  patient  himself  had  no  knowledge  of  thèse  absences,  the  existence  of 
which,  indeed,  he  was  disposed  to  deny. 

One  evening  towards  seven  o'clock,  I  was  sent  for  in  ail  haste.  M.  A —  had 
been  stricken  two  hours  before,  during  an  absence,  the  duration  of  which  had 
not  exceeded  the  usual  time,  with  sudden  paralysis  affecting  the  left  upper 
extremity.  On  coming  to  his  sensés,  he  found  his  limb  quite  inert,  flaccid, 
hanging  by  his  side.  I  ascertained  that  the  paralysis  was  limited  to  the  left 
upper  extremity,  which  it  occupied  throughout,  and  it  affected  neither  the  face, 
nor  the  corresponding  lower  extremity.  The  persons  présent  assured  me  that,  at 
no  time,  had  anything  resembling  convulsions  occurred.  Nor  was  there  any  dis- 
turbance  of  sensibility  in  the  paralysed  part  ;  neither  ansesthesia,  nor  aualgesia, 
nor  formication.  The  monoplegia  progressively  improved,  during  the  evening, 
in  a  very  rapid  manner.     On  the  morrow  morning  no  trace  of  it  remained. 

The  différent  circumstances  just  related  struck  me  very  vividly  ;  the  in- 
fluence of  syphilis  seemad  to  me  no  longer  susceptible  of  being  misunderstood  ; 
I  immediately  prescribed  the  mixed  treatment,  according  to  the  method  which 
bas  been  several  times  mentioned  in  the  course  of  the  présent  lecture. 

A  new  attack  of  left  brachial  monoplegia,  similar  in  ail  respects  to  the  pre- 
ceding  one,  which  did  not  last  longer  than  from  four  to  five  hours,  took  place 
three  or  four  days  after  the  beginning  of  the  treatment  :  it  was  the  last.  The 
cephalalgia,  the  ansemia,  the  prostration  of  strength,  ail  thèse  also  vanished 
with  marvellous  rapidity,  in  such  a  way  as  to  demonstrate  to  the  most  incredulous 
that  I  had  now  struck  home.  The  treatment  was  contiuued,  with  the  inter- 
ruptions ordered,  for  nearly  three  months. 

Five  or  six  months  ago  I  received  news  of  M.  A — ,  and  was  happy  to  learn 
that,  from  the  time  I  lost  sight  of  him,  no  symptoms  of  the  disorder  had 
reappeared. 


300  LOCALISATION   OF   LESIONS. 

anatomical  substratum  of  syphilitic  partial  epilepsy.  They  hâve 
been  already  described,  with  a  certain  exactuess^  in  one  observation 
relating  to  this  affection,  published  by  Todd,  in  1857.^  Twa 
chromo-lithographie  plates,  annexed  to  the  work  of  M.  G.  Eche- 
verria,^  which  give  a  faithful  représentation  of  thèse  lésions, — not 
often  to  be  met  with  in  post-mortem  sections, — likewise  concern  a 
case  of  partial  epilepsy.  The  same  is  true  of  a  design  by  Lacker- 
bauer,  published  by  M.  Lancereaux,  in  his  '  Traité  de  la  Syphilis.'^ 
Unfortunately,  the  clinical  observations  which  thèse  figures  are 
intended  to  illustrate  leave  much  to  be  desired. 

Besides  partial  epilepsy,  very  différent  clinical  forms  of  cérébral 
syphilis  may  also  dépend  on  gummatous  pachymeningitis.  It  is 
no  longer  doubtful  to-day  that  there  are  often  deep  différences  in 
the  symptomatic  expression  of  the  same  organic  altération,  which 
especially  dépend  on  its  mode  of  localisation  on  the  surface  of  the 
hémisphères.  According  to  a  theory,  founded  on  récent  work,  the 
gummatous  patches,  in  partial  epilepsy,  ought  to  be  situated  on 
the  surface  of  the  frontal,  or  ascending  pariétal  convolutions,  or,  at 
ail  events,  in  their  immédiate  neighbourhood.  The  reality  of  this 
statement  has  not  yet  been  regularly  verified,  so  far  as  I  am  aware, 
up  to  the  présent  ;  but  it  will,  doubtless,  not  long  remain  so.  In 
the  meanwhile  I  may  point  out  that,  in  the  plate  of  Echeverria, 
just  mentioned,  it  is  easy  to  recognise  that  the  gummatous  lésions 
of  the  pia  mater  occupied  the  immédiate  neighbourhood  of  Rolando's 
fissure,  behind  it,  not  far  from  the  médian  fissure,  that  is  ta 
say,  a  région,  in  part,  belonging  to  the  domain  of  the  cortical  motor 
zone. 

So  long  as  the  syphilitic  partial  epilepsy  is  not  inveterate,  so  long 
as  the  attacks  which  constitute  it,  clinically,  remain  separated  by 
intervais  free  frora  ail  permanent  symptoms,  the  cérébral  grey  sub- 
stance, in  contact  with  the  altered  pia  mater,  has  as  yet  suffered,  as 
everything  leads  us  to  believe,  only  lésions  of  a  kind  which  has 
sometimes  been  called  dynamic — transient  lésions  at  most,  and  not 
disorganising  ones.  In  such  cases  there  would,  according  to  H. 
Jackson,  be  produced  in  the  nerve  matter,  owing  to  an  irritative 
process  determined  by  vicinity,  a  sort  of  storing-up,  an  accumula- 
tion of  energy  which  would  be  expended  from  time  to  time,  under 

^  'Médical  Gazette,'  Jarmary,  1851,  and  'Clinical  Lectures,'  loc.  cit. 
^  'On  Epilepsy,'  New  York,  1870,  pi.  iii,  and  6. 
^  Paris,  1866,  pi.  ii,  fig.  vi. 


NECESSITY   FOR  ACTIVE  INTERVENTION.  301 

the  influence  of  tlie  most  trivial  and  often  imperceptible  causes,  in 
a  sort  of  explosion  of  sudden,  convulsive,  and  disorderly  motor  acts, 
bearing  upon  the  side  of  the  body  opposite  to  the  meningeal  lésion. 
The  discharge  would  always  be  folio wed  by  raomentary  exhaustion, 
which  is  clinically  translated,  by  the  temporary  paralysis  with 
flaccidity,  which  is,  in  truth,  very  frequently  observed  after  attacks 
of  partial  epilepsy,  in  those  very  parts  which  hâve  been  the 
principal  seat  of  the  convulsions.  If  this  be  not,  properly  speaking, 
a  regular  theory,  it  is  at  least  an  ingénions  manner  of  grouping  the 
facts. 

In  the  course  of  time,  owing  to  the  répétition  of  thèse  acts,  or 
else  because  of  the  progressive  extension  of  the  meningeal  lésions 
to  the  nerve  substance,  the  latter,  in  its  turn,  becomes  gravely 
altered  ;  then,  at  the  same  time  when  descending  secondary  de- 
generations  are  produced,  permanent  and  indelible  hemiplegia  may 
supervene.^ 

Thèse  anatomo-pathological  and  physiological  considérations 
concur,  as  you  see,  to  emphasise,  once  again,  the  importance  of 
prompt  and  energetic  décisions  when  dealing  with  partial  epilepsy 
of  syphilitic  origin.^ 

^  On  the  production  of  secondary  degenerations  consécutive  on  lésions  of 
the  cortical  motor  zone,  see  Charcot  :  '  Leçons  sur  les  localisations  dans  les 
maladies  du  cerveau,'  p.  i6o,  Paris.  1876.  M.  Hauot,  four  or  five  years  ago, 
presented  to  the  Société  Anatomique,  an  example  noted  iu  M.  Charcot's  wards, 
of  descending  degeneration,  with  permanent  hemiplegia,  consécutive  on  a 
gummatous  lésion  of  the  brain. 

2  Consult  also,  "  A  Case  of  Syphilitic  Disease  of  the  Brain,"  by  J.  Dresch-. 
feld,  'Laucet,'  1877,  vol.  i,  p.  268.  This  observation  is  peculiarly  interestiug 
from  the  point  of  view  of  lésion  localisation. 


APPENDIX. 


I. 

MULTIPLE  PATHOLOGICAL  LUXATIONS  AND  SPONTANEOUS 
ERACTURES  IN  A  PATIENT  SUEEERING  EROM  LOCO- 
MOTOR  ATAXIA.    By  J.  M.  Charcot. 

(See  Lecture  IV,  p.  47.) 

The  case,  of  which  I  am  about  to  state  the  détails,  présents  a 
new  example  of  tliose  tropJiic  disorders  of  the  peripheral  parts, 
produced  in  conséquence  of  a  lésion  of  the  nerve-centre,  to  which 
I  hâve  called  the  attention  of  physiologists  and  physicians.  The 
question  hère  relates  to  multiple  arthropathies  and  spontaneous 
fractures  in  the  case  of  a  woman  attacked  with  progressive  loco- 
motor  ataxia. 

Observation.' — Progressive  locomotor  ataxia.  Consécutive  luxa- 
tions and  spontaneous  fractures.  Complète  ilio-jjitbic  luxation 
of  the  left  coxo-femoral  articulaiion.  Shortening  of  left 
fémur.  Fracture  of  the  anatomlcal  nech  of  the  left  fémur. 
Complète  ilio-ischiatic  luxation  of  the  right  coxo-femoral  arti- 
culation. Complète  sub-coracoid  luxation  of  the  left  scapulo- 
kumeral  articulation.  Consolidated  fracture  with  deformed 
and  oblique  callus  ofboth  bones  of  the  leftfore-arm.  Chronic 
arthritis  of  the  right  scapulo-humeral  articulation.  Consoli- 
dated fractures  with  voluminous  callus,  of  hoth  bones  of  the 
right  for  e-arju?- 

The  patient,  A.  Co— t,  aged  57,  domestic  servant,  was  admitted 
to  the  Salpêtrière,  as  an  invahd,  February  8th,  1866.  She  entered 
theinfirmary  of  the  Asylum,  Salle  St.  Jacques^No.  23,  Oct.15, 1873. 

This  woman  had  nine  children  ;  seven  of  them  died  between  the 
âges  of  five  and  fifteen  months.  Another  succumbed  at  the  âge  of 
thirty-four,  after  childbirth.  There  is  nothing  to  note  in  the  history  of 

'  Edited  from  notes  takeu  by  M.  Bourneville.  See  also,  Eorestier,  'Thèses 
de  Paris;  1874. 

VOL.  II.  20 


306       LOCOMOTOE  ATAXIA.       SPONTANEOUS    LUXATIONS. 

tlie  patient,  if  not  some  violent  megrims,  accompanied  by  vomiting, 
usually  coinciding  witli  the  menses,     The  megrims  disappeared  to  a 
great  extent  when  slie  was  about  thirty-five  (1850) — at  which  period, 
the  lightning  pains,  which  marked  the  onset  of  the  présent  disease, 
made  their  appearance.     Thèse  pains  occupied  the  lower  hinbs,  first 
of  allj   especially   the  calves,  and  the  instep.     "  I  felt,"  she  said, 
"  like  lightniugs  going  through  my  legs,"  The  pains  were  violent, 
more  severe  by  night  than  by  day,  coming  on  in  paroxysms,  which 
lasted  for  about  from  twelve  to  fifteen  hours.     Towards  the  same 
period,  a  constant  feeling  of  painful  constriction  at  the  base  of  the 
chest  supervened.     The  painful  crises  which,  in  the  early  stage, 
showed  themselves  about  every  three    weeks   became  afterwards 
more  fréquent  and  more  violent.  They  apppear  to  hâve  reached  their 
maximum  of  intensity  and  frequency  towards  the  âge  of  thirty-eight. 
At  the  âge  of  forty-two,  after  sensations  of  numbness  in  the 
right  foot,  the  patient  one  day  remarked  tliat  the  fïgM  thigh  was 
conslderahly  swollen  ;  the  limb,  hère,  it  would  appear  had  nearly 
doubled  in  size.     This  swelling  was  accompanied  neither  by  redness 
nor  by  pain  ;  it  did  not  hinder  C —  from  contiuuing,  as  before,  and 
without  marked  hindrance,  her  work  as  a  servant.     The  sw^elling 
and   numbness   remained  for   several   mouths.     Thèse  symptoms 
were  improving,  when,  one  morning  (1858),  on  getting  out  of  her 
bed,  the  patient  remarked  witli  astonishment,  that  she  was  lame, 
and  that  her  right  lower  extremity  was  shorter  than   the   left. 
During  the  night,  in  bed,  and  without  pain,  luxation  of  the  right 
hip  had  taken  place. 

Walking,  from  this  time,  became  difficult,  but  not  impossible, 
far  from  it  ;  for  C — ,  being  unable  to  continue  to  act  as  a  servant, 
was  still  able,  for  nearly  a  year,  to  proceed  daily  ou  foot  to  a 
distant  "  Hôtel  meublé,"  where  she  was  employed  to  make  the  beds. 
Towards  the  commencement  of  the  year,  1859,  there  supervened 
in  the  left  foot  a  numbness  similar  to  that  which  had,  previously, 
occupied  the  right.  This  numbness  remained  for  several  months 
when,  one  night,  in  conséquence  of  a  movement  whilst  in  bed,  a 
crack  was  heard  in  the  left  hip,  which  became  liuvated. 

Prom  this  day,  both  the  lower  extremities  being  alike  shortened, 
ail  active  work  became  henceforth  impossible.  C —  had  herself 
taken  to  the  Hôpital  de  la  Charité,  where  she  remained  for  four 
months.  At  this  time,  she  could  stand  up,  and  even  walk,  by 
leaning  against  the  walls  as  she  went  ;   she  experienced,  as  in  the 


SPONTANEOUS  PEACTUEES.  307 

pastj  paroxysms  of  lightning  pains  in  the  lowerlimbs.  Thèse  pains 
liad  not  yet  sliown  themselves  in  the  upper  extremities. 

After  leaving  La  Charité,  C —  was  successively  aclmitted  into 
several  hospitals;  lastly,  she  was  received,  July,  1865,  into  the 
Hôtel  Dieu,  M.  Yigla's  Ward.  At  this  period,  the  patient  had 
the  full  use  of  her  upper  extremities,  which  were  not  affected  by 
any  pain  ;  as  to  her  lower  extremities  when  the  lightning  pains 
continued  to  appear  from  time  to  time,  she  could,  whilst  in  bed, 
move  them  about  strongly,  but  thèse  movements  had  become 
irregular,  ill-coôrdinated,  recalling  those  of  the  legs  of  a  puppet.  The 
several  joints  of  thèse  limbs  had  acquired  an  extrême  laxity  ;  thus 
C —  could  easily  '^embrace  her  foot,"  even  put  it  behind  her  head, 
things  which  it  had  been  impossible  for  her  to  do  formerly.  One 
day,  whilst  in  bed,  and  wishing  to  show  off  her  suppleness,  she 
brought  her  left  foot  towards  her  mouth  as  if  to  embrace  it,  and  in 
this  movement  the  \di  femw'  fractured. 

The  lightning  pains  commenced  to  appear  in  the  upper  extre- 
mities about  a  year  after  her  admission  to  the  hospice  of  La  Salpê- 
trière  (end  of  1866),  and  since  then  they  hâve  not  ceased  to 
supervene  in  crises.  Sometimes  they  are  felt  in  one  spot,  some- 
times  in  another  ;  they  are  never  so  violent  as  those  which,  from 
time  to  time,  still  invade  the  lower  extremities.  The  movements 
of  the  upper  limbs  were  not  affected  until  lately.  Thus,  at  the  end 
of  June,  1873,  C —  could  still,  without  difficulty,  lift  her  food  to 
her  mouth,  do  needlework,  pick  up  from  her  bed  the  smallest  ob- 
jects,  a  needle,  a  bit  of  thread.  However,  she  sometimes  experienced 
a  stiffness  in  her  fingers,  which  stretched  out  stubbornly.  This  ex- 
cepted,  no  trace  of  motor  incoordination  existed  in  the  upper 
extremities  at  that  date. 

Towards  the  middle  of  July,  1873,  C — ,  whilst  she  was  changing 
her  position  in  htà.,  fractured  hoth  boues  of  the  left  for earm.  She 
could  not  tell  exactly  how  this  fracture  came  about,  but  it  is 
certain  that  there  was  neither  a  fall,  nor  any  violent  effort.  No 
apparatus  was  applied.  Consolidation  took  place  rapidly;  the 
callus  is  voluminous  and  deforraed.  Since  the  time  of  the  ac- 
cident, the  left  hand  présents  a  peculiar  déformation  which  tends 
each  day  to  grow  worse.  The  thenar  and  hypothenar  eminences 
are  approximated  each  to  each  ;  the  thumb  stretched  out  rests  on 
the  index  which  is  slightly  flexed.  The  other  fingers  are  likewise 
in  deraiflexion. 


308  LOOOMOTOR   ATAXIA. 

Three  montlis  after  (September,  1873),  at  the  time  when  C — 
was  helping  herself  to  sit  up  in  bed,  with  her  right  hand,  the  riffài 
forearm  fractiired,  in  its  turn,  about  rnidway.'  No  pain  was  felt 
when  tlie  fracture  liappened.  An  apparatus  was  adjusted  to  the 
arm.  Consolidation  took  place  rather  promptly  without  much 
dif&culty.  The  right  hand  begins  to  exhibit  a  déformation  analo- 
gous  to  that  presented  by  the  left  hand.  The  movements  of  the 
différent  segments  of  the  members  are  still  easy  and  regular.  C — 
can  carry  her  food  to  her  mouth  with  her  right  hand,  pick  up  small 
objects,  &c.  Pinally,  October  iith,  1873,  after  some  insignificant 
movement,  a  luxation  of  the  left  shoulder  occurred.  The  shoulder 
had  exhibited  no  preliminary  swelling  ;  and  the  patient  had  felt  no 
pain  in  it. 

Towards  the  same  period,  visual  disorders  were  remarked,  for  the 
first  time  ;  now  and  again,  objects  appeared  double  ;  sparks  some- 
times  passed  before  the  eyes.  It  is  noted  that  the  right  pupil  is, 
permanently,  more  dilated  than  the  left. 

Présent  state  (noted  26th  November,  1873). — ^^  ^J  request, 
my  colleague  Dr.  Meunier,  Surgeon  to  La  Salpêtrière,  has  been 
kind  enough  to  study  and  describe  with  great  care  the  lésions  pre- 
sented by  the  joints  and  bones  in  this  case  of  C — .  I  reproduce, 
in  extenso,  the  note  which  he  has  obligingly  made  in  référence  ta 
this  subject. 

"  Left  lower  extremity. — It  is  much  shortened,  and  measures  62 
centimètres  from  the  anterior  and  superior  iliac  spinous  process  to  the 
external  malleolus.  It  is  placed  on  its  external  side  in  abduction 
and  rotation  outwards.  There  is  no  noticeable  pecuKarity  to  be  re- 
marked  as  regards  the  foot,  the  leg,  the  knee,  or  even  the  lower 
part  of  the  thigh.  It  is  the  upper  part  of  the  thigh  and  hip,  and, 
to  be  exact,  it  is  the  fémoral  articulation  which  isinjured.  The  tro- 
chanter  major  is  depressed,  and  in  such  a  position  that  it  is  carried 
towards  the  postero-external  portion  of  the  thigh.  It  is  situate  at 
a  distance  of  about  12  centimètres  from  the  anterior  superior  iliac 
spinous   process,    when    the    member    is   brought    straight    (see 

Plate  VI). 

"  Ail  the  internai  part  of  the  thigh,  from  8  centimètres  above  the 
internai  condyle  of  the  fémur,  présents  a  succession  of  transverse 
folds,  more  or  less  deep,  to  the  number  of  about  a  dozen  :  some 
occapy  the  whole  of  this  internai  part,  even  going  beyond  it,  at  the 
back  j  others,  uearer  to  the  groin,  pass  across  and  beyond  the  an.^ 


SPONTANEOUS    LUXATIONS.  309 

terior  part  of  the  thigli,  extendiiig  even  towards  the  external  latéral 
part,  but  not  reacliing  it. 

"On  the  external  part  of  tlie  tliigli  are  noticed  some  rolls  of 
skin^  in  the  longitudinal  direction  of  the  limb — rolls  wiiich  are 
efFaced  on  movement. 

"  The  forin  of  the  thigh  is  that  of  a  truncated  cône.  The  entire 
thigh  is  shortened,  and  measures,  from  the  anterior  superior  iliac 
spinous  process  to  the  internai  condyle  of  the  femur^  a  distance  of 
23  centimètres. 

"The  hip-joint  possesses  its  six  movements  :  flexion,  extension, 
adduction,  abduction,  rotation,  and  circumduction.  The  motion  of 
extension  is  limited.  The  movement  of  abduction  is  most  extended  ; 
the  leg  and  the  thigh  can  be  wholly  placed  ou  their  external  latéral 
part  where  they  lie  on  the  bed-surface.  Thèse  are  the  movements 
performed  by  the  patient  herself,  One  can  produce  them  as  com- 
j)letely  as  described  above.  In  assisted  movements,  especially 
when  the  limb  is  rotated  outwards,  well-marked  cracking  sounds 
are  heard,  doubtless  owing  to  the  rubbing  of  two  rough  surfaces. 

"  We  infer  from  the  above  description  that  there  is  a  luxation  of 
the  Jiip.  The  head  of  the  fémur  being  carried  upwards  and  in- 
wards,  the  variety  of  luxation  is  that  designated  by  the  name  of 
ilio-pubic  luxation;  let  us  add  that  this  luxation  is  co7)iplete,  and 
due  to  a  patJiological  cause. 

"  The  fémur,  very  much  shortened,  exhibits  no  altération  in  its 
lower  part,  nor  in  its  médian  part,  up  to  and  inclusive  of  the  tro- 
•chanter  major.  In  ail  this  part  of  the  diaphysis  of  the  bone,  there 
is  neither  solution  of  continuity  nor  augmentation  of  size.  It  is 
otherwise  higher  up,  where  we  note  impotence  of  the  limb,  which 
cannot  be  raised  completely  from  the  bed-level,  especially  the  lieel. 
At  the  root  of  the  limb,  also,  abnormal  mobility  exists,  witli  crépi- 
tation— characters  indicative  of  fracture  of  the  anatomical  neck  of 
the  fémur  :  consequently,  intra-articidarfractv,re;  such  are  the  most 
marked  ratioual  and  perceptible  signs  observed  on  examination. 

"  B,igh,t  lower  limb. — Its  length  is  74  centimètres,  and,  therefore, 
12  centimètres  more  than  that  of  the  opposite  sjde  :  this  length  is 
measured  from  the  anterior  superior  iliac  spinous  process  to  the 
external  malleolus.  The  limb  is  placed  in  rotation  inwards,  the 
knee  and  internai  border  of  the  foot  toucliing  the  bed.  Less 
numerous  but  more  oblique  folds  or  wrinkles  than  were  seen  on  the 
other  extremity  are  found  on  the  internai  part  of  the  thigli  from 


310  LOOOMOTOll   ATAXIA. 

tlie  inner  border  of  the  knee  to  the  groin.  Six  or  seven  rather 
marked  folds  may  be  counted,  extending  from  the  knee  and  from 
the  internai  part  of  the  thigh  to  within  a  few  centimètres  of  the 
anterior  superior  iliac  spinous  process.  Thèse  wrinkles  extend 
obliquely  from  below  upwards,  from  within  outwards^  in  an  oblique 
and  almost  vertical  direction.  The  trochanter  major  isbrought  up, 
aud  placed  on  a  line  whicli  may  be  supposed  to  unité  transversally 
the  anterior  superior  iliac  spinous  process  to  the  ischium. 

"  The  several  movements  of  the  right  coxo-femoral  articulation 
may  be  performed  by  the  patient  herself.  They  may  also  be  prac- 
tised  by  the  observer  ;  but  whilst  the  movements  of  adduction  and 
flexion  are  exaggerated,  on  the  other  hand_,  the  movements  of  ab- 
duction and  rotation  outward  are  limited  and  even  markedly 
diminished.  It  is  whilst  producing  thèse  movements  that  the  sensa- 
tion of  cracking  is  experienced  in  the  coxo-femoral  articulation. 
The  fémur,  throughout  its  entire  length,  and  at  both  extremities,  ex- 
hibits  no  solution  of  continuity,  and  no  augmentation  of  volume; 
the  head  of  the  fémur  can  be  easily  felt  through  the  skin,  from  the 
external  part  of  the  hip.  It  is  carried  directly  backwards  and 
upwards.     Hence  there  is  an  ilio-ischiatic  luxation. 

"  TJpper  left  limb. — It  présents  lésions  in  the  shoulder  and  fore- 
arm.  From  the  acromion  to  the  epitrochlea  the  arm  measures  31 
centimètres.  It  is  consequently  somewhat  lengthened.  The 
shoulder  itself  is  manifestly  flattened.  The  anterior  wall  of  the 
axillary  hoUow  exhibits  several  vertical  wrinkles.  The  several 
movements  of  the  scapulo-humeral  articulation  are  spontaneously 
produced,  with  abnormal  vivacity,  in  conséquence  of  the  ataxia 
which  affects  the  patient.  Thèse  movements  may  likewise  be  per- 
formed by  the  observer  ;  the  movement  of  élévation  of  the  arm  is 
the  most  limited  of  auy  ;  the  movements  of  adduction  are  dimin- 
ished, the  patient  not  being  able  to  completely  bring  the  arm  close 
to  the  body  ;  the  signs  of  the  existing  luxation  are  not  very  marked  ; 
liowever,  we  flnd,  on  pressing  the  axillary  cavity  with  the  fingers, 
that  the  head  of  the  humérus  is  approximated  to  the  internai  part 
and  depressed.  From  this  array  of  signs  we  conclude  that  there 
exists  a  complète  suhcoracoid  luxation.  The  crépitation  is  weU. 
marked  in  the  movements.  The  remainder  of  the  humérus  is 
Sound. 

*'The  left  forearm  appears  slightly  shortened;  it   shows  aug- 
mentation of  size  in  its  upper  third.     There,  in  fact^  we  find  a 


SPONTANEOUS    FRACTURES    AND    LUXATIONS.  311 

slïghthj  (leformed  câlins  occupying  hotli  houes,  beginning  at  the 
upper  third  of  the  ulua,  nearly  6  centimètres  from  the  olecranon, 
and  proceeding  down  the  forearm  towards  the  radius.  This  callus 
describes  a  curve  with  convexity  in  front,  and  concavity  behind.  It 
extends  to  nearly  4  centimètres  from  the  styloid  apophysis  of  the 
radius.  It  occupies  ail  the  interosseous  space  towards  the  upper  third 
of  the  forearm,  especially  behind.  This  voluminous  callus,  elon- 
gated  from  above  downwards,  from  within  outwards,  indicates  an 
old  Consolidated  fracture.  This  spontaneous  fracture  affected  both 
bones,  the  ulna  at  its  upper  third,  and  the  radius  in  its  middle 
portion  ;  that  is  to  say,  that  there  was  hère  an  oblique  fracture  of 
the  forearm.  The  callus,  just  described^  greatly  interfères  with  the 
movements  of  flexion  and  extension  of  the  fingers.  The  fingers 
of  the  left  hand  are  habitually  stretched,  but  extension  and  flexion 
can  be  completely  executed;  still,  in  their  usual  condition,  the 
fingers  are  placed  in  relatively  différent  positions,  the  index  being 
permanently  stretched. 

"  Right  upper  limb. — Its  length,  measured  from  the  acromion  to 
the  epitrochlea,  is  29  centimètres  ;  it  is,  consequently,  shorter  by 
2  centimètres  than  that  of  the  opposite  side  ;  there  is  no  luxation 
of  the  shoulder.  The  movements  of  the  scapulo-humeral  articula- 
tion are  ail  made  in  their  entirety,  only  at  times  some  cracks  are 
heard  on  moving,  indicating  incipient  arthritis. 

"  The  forearm  exhibits  lésions  analogous  to,  if  not  identical  with, 
those  described  above  for  the  left  forearm.  A  voluminous  callus 
exists,  which  we  are  about  to  describe.  This  callus  indicates  a 
fracture  involving  hoth  bones  near  and  below  their  middle  part.  The 
larger  callus  is  that  of  the  ulna  ;  it  is  situate  on  the  inner  border 
of  this  bone.  This  callus  is  about  4  centimètres  long,  and  descends 
to  about  the  same  length  from  the  inferior  portion  of  the  bone.  It 
is  about  3  centimètres  in  thickness  ;  much  less  in  length  is  the 
callus  of  the  external  side,  that  is  to  say,  that  of  the  radius  ;  its 
thickness  may  be  regarded  as  perceptibly  the  same  as  that  on  its 
companion  bone.  The  movements  of  the  fingers,  namely,  those 
produced  by  the  action  of  the  flexor  and  extensor  muscles  of  the 
digits,  are  much  more  easily  made  than  in  the  opposite  extremity. 
This  greater  facility  of  motion  must  be  attributed  to  the  conditions 
in  which  we  tind  the  forearm. 

"In  conclusion,  there  was  complète  fracture  ofboth  bones,  which 
is  now  Consolidated  and  présents  a  voluminous  callus^' 


31,2  LOCOMOTOR  ATAXIA. 

I  sliall  complète  tliis  statement  by  some  détails  relating  especially 
to  différent  sensorj  ancl  motor  disorclers,  and  to  the  gênerai  state  of 
tlie  patient. 

Digestive  apparatus. — The  tongue,  wlien  protruded,  is  stirred  by  a 
slight  trembling,  cliiefly  marked  at  the  tip  on  the  right  sidc.  The 
appetite  is  good.  Déglutition  easy.  No  difficulty  of  défécation. 
The  patient  several  times  experienced  lightning  pains  occupying  the 
inferior  part  of  the  rectum  and  labia  majora.  Thèse  pains  are,  gene- 
rally,  less  intense  than  those  which  are  felt  in  the  limbs. 

Clrculatioii  and  respiration. — Puise  small,  regular,  middling 
fréquent,  heart  normal.  Auscultation  and  percussion  give  négative 
results^  with  respect  to  lungs. 

TJrinary  function  normal. — The  urine  exhibits  no  altération  in  its 
physical  and  chemical  constitution. 

During  the  course  of  late  years  C —  has  suffered  considérable 
emaciation.  She  formerly  measured  85  centimètres  around  the 
waist  ;  to  day,  she  measures  no  more  than  64.  This  emaciation  has 
made  especial  progress,  since  1868. 

The  ribs  are  solid  and  do  not  give  way  at  ail  to  pressure,  as 
usually  occurs  in  osteomalacia.  Neither  the  pelvis,  nor  the  fingers, 
exhibit  the  Reformations  usually  connected  with  this  affection. 

Moveuents,  sensibility . — It  is  found  that  the  movements  of  the 
lower  extremities,  limited  though  they  be,  are  still  rather  energetic  ; 
but  they  are  mauifestly  incoordinate;  moreover,  the  patient  has 
completely  lost  sensé  of  the  positions  in  which  her  limbs  may  be 
placed.  Provoked  as  well  as  spontaneous  movements  are  effected 
completely  without  pain. 

On  the  limbs,  the  patient  feels  touch,  tickhng,  pinching,  pin- 
pricking.  There  is  no  perceptible  différence  in  this  respect  between 
the  two  lower  members.  However,  it  appears  manifest  that  sensi- 
bility  is  markedly  blunted  in  the  feet.  Examination  of  sensibility 
to  cold  furnished  the  following  results  :  If,  after  closiug  the  patientas 
eyes,  a  pewter  vessel  be  applied  to  the  différent  segments  of  the 
lower  limbs,  sometimes,  a  burning  sensation  is  perceived,  sometimes 
the  touch  merely.  Neither  the  cold  nor  the  weight  of  the  vessel  is 
felt.  But  if  duriûg  the  experiment  the  patient  be  permitted  to  look, 
she  succeeds,  after  making  a  kind  of  effort  to  get  at  the  true  sen- 
sation, in  recognizing  that  she  is  at  first  deceived  and  that  the  object 
touching  her  is  really  cold. 

The  patient  cannot  at  présent  make  use  of  the  left  uj)per  ex~ 


ARTHROPATHIES  AND   FEACTURES.  313 

tremity^  even  to  help  in  feedmg  herself.  She  has  little  strength  in 
the  hand  on  this  side,  and  the  raotor  incoordination  is  well-marked 
throughout  the  limb,  whether  the  eyes  be  shut  or  open,  when  she 
is  executing  the  movements.  The  motor  incoordination  has  shown 
itself  especiallv  during  the  past  month.  The  différent  modes  of 
sensibility  are  preserved  hère.  Hovvever,  the  patient  does  not  dis- 
tinctly  perceive  the  différence  between  two  bodies  unequally  cold. 
The  movements  of  the  right  upper  extremity  are  less  gravely 
affected  ;  incoordination  is  less  marked  ;  it  is  notabîy  increased  when 
the  eyelids  are  closed.  To-day  she  cannot  raise  a  glass  to  her  lips 
with  this  hand,  except  with  great  difïiculty. 

December,  1876. — The  crackiug  sounds  in  the  right  shoulder  are 
becoming  more  and  more  manifest  ;  no  pain  nor  swelling  is  observed 
at  the  joint. 

December  I5th. — C —  has  remarked,  for  some  days,  that  she 
expériences  crackings  in  the  left  teynporo-maxlllary  articulation.  It 
is  observed  that  the  articulation  in  question  possesses  exaggerated 
mobility,  spontaneous  or  provoked  movements  do  not  cause  any 
pain.^ 

^  This  patient  died  in  1S76,  of  rupture  of  an  aortic  aneurism.  Tlie  spinal 
cord  sliowed  sclerosis  of  the  posterior  columns  characteristic  of  the  locomotor 
ataxia.  The  lésions  on  whicli  we  hâve  liere  to  insist  are  those  of  the  hones  aud 
of  the  joinis. 

The  two  scapulae  which  were  fractured  are  shoitened,  because  the  iuferior 
segment  has  slipped  up  on  the  posterior  surface  of  the  superior  segment.  They 
exhibit  a  callus,  regular  enough  and  complète  on  the  right — irregular  and  in- 
complète on  the  left,  inasmuch  as  the  fracture  which  has  the  form  of  a  right 
angle  with  apex  internally,  is  only  Consolidated  in  its  vertical  portion — the  two 
lips  of  the  horizontal  portion  of  the  fracture  are  not  united  and  are  covered 
with  osseous  végétations.  The  fractures  are  situated  at  the  middle  part  of  the 
sub-spinous  fossa. 

The  le/t  clavide  exhibits  a  Consolidated  fracture,  situated  at  the  junction  of 
its  external  fourth  with  its  internai  three  fourths. 

The  rif/ht  ulna  and  radius  présent  voluminous  calluses  consécutive  on 
fractures  which  took  place  at  the  junction  of  the  inferior  third  with  the  middle 
third.     Both  are  shortened. 

On  the  left  ulna  is  found  a  very  large  callus  at  the  junction  of  the  upper  fourth 
with  the  lower  three  fourths.  From  the  external  and  posterior  border  of  this 
callus  starts  an  osseous  jetty,  which  proceeds  obliquely  dowuwards,  describing 
a  curve,  concave  behind,  and  unités  with  the  radius,  a  little  below  its  upper 
third.  The  radius  which  was  fractured,  in  its  middle  portion,  présents  a  callus, 
five  or  six  centimètres  in  length,  voluminous,  and  which  gives  origin  below  to 
another  osseous  jetty,  which  proceeds  to  unité  with  the  anterior  surface  of  the 
ulna,  in  such  a  manner  as  that  the  superior  jetty  is  situated  behind  the  two 


314  LOCOMOTOE   ATAXTA. 

Tliis  observation,  I  think,  does  not  require  a  long  commentary  j 
tlie  lessons  whicli  it  contains  speak  for  themselves. 

Tlie  peculiar  circumstances  in  which,  under  tlie  influence  of  quite 
insignificant  traumatic  causes,  the  several  fractures  were  produced, 
alike  in  the  thigli  and  in  the  two  forearms,  do  not  permit  us  to 
recognise  in  this  case  the  intervention  of  any  of  the  influences  which, 
as  a  rule,  préside  over  the  development  of  i\\t  fractures  callecl  spon- 
taneous.  Thus,  for  instance,  we  had  grounds  for  eliminating  ail 
action  of  hereditary  prédisposition,  and  again  ail  influence  of  a 
diathetic  élément,  such  as  syphilis,  cancer,  goût,  rheumatism.  I 
will  add  that  the  différent  parts  of  the  skeleton,  particularly  the 
ribs  and  the  pelvic  bones,  do  not  présent  any  of  the  lésions  which, 
clinically,  may  serve  to  mark  the  disease  known  as  osteomalacia. 

Lastly,  and  this  point  deserves  particularly  prominence,  we 
cannot  invoke  the  existence  of  a  nutritive  disorder  of  the  osseous 
tissue  resulting  from  a  prolonged  functional  inaction  of  the  limbs, 
itself  consécutive  on  the  spinal  affection.  Ail  the  détails  of  the 
observation,  on  the  contrary,  establisli  clearly,  so  far  as  the  upper 

bones,  and  the  lower  one  on  the  contrary,  anterior  to  them.  On  both  sides,  the 
bones  of  the  fore-arm  are  shortened,  the  radius  principally,  and  the  left  radius 
in  particular  (Plate  IX). 

ïhe  coxo-femoral  articulations  exhibit  the  usual  lésions  of  the  arthropathies 
of  ataxic  patients.  Right  and  left,  the  border  of  the  cotyloid  cavities  which  is 
so  marked  in  the  normal  state,  is  mostly  effaced,  it  has  even  disappeared  iu 
the  lower  half,  especially  on  the  right.  In  fact,  on  this  side,  the  cotyloid 
cavity  becomes  confounded  with  the  external  surface  of  the  ischium.  Above, 
the  cotyloid  cavity  is  not  more  than  a  centimètre  in  depth,  and  hardly  a  centi- 
mètre and  a  half  on  the  left. 

The  lésions  are  still  more  marked  as  regards  the  femiir s.  On  the  right,  the 
head,  the  neck,  and  a  considérable  portion  of  the  trochanter  major  hâve 
disappeared.  On  the  left,  the  head  no  longer  exists,  the  neck  persists  but 
reduced  to  a  rudimentary  state,  reduced  in  size  by  two  thirds  ;  the  trochanter 
major  is  worn  down,  and  what  remains  of  the  upper  extremity  of  the  fémur 
terminâtes  in  an  irregular  callus,  presenting  in  its  inferior  and  interior  part 
a  sort  of  triangular  jetty  in  the  form  of  a  lamina  separated  from  the  corre- 
sponding  surface  of  the  bone  by  an  interval  of  three  or  four  millimètres. 
In  a  Word,  besides  the  lésion  due  to  the  arthropathy,  we  hâve  hère  a  fracture. 
Neariy  the  whole  of  the  upper  fragment  is  destroyed  by  atrophy,  by  rubbing, 
and  the  remaining  portion  is  united  with  the  inferior  fragment.  From  the 
lésions  just  described  it  also  results  that,  whilst  the  right  fémur  is  50  centi- 
mètres in  length,  the  left  is  only  19  centimètres  (Plate  X). 

It  will  be  seen,  by  the  preceding  description,  and  thèse  are  very  interesting- 
lésions,  of  a  kind  altogether  peculiar,  that  are  not  to  be  met  with  in  the  ordi- 
nary  forms  of  dry  arthritis. — B. 


SPONTANEOUS   FEACTUEES.  SIS- 

limbs  are  concerned,  that  the  fractures  occurred  at  a  period  when 
thèse  limbs  still  possessed  ail  tlieir  physiological  movements,  the 
spinal  disease  being  hère  represented  by  shooting  pains  only  ;  and, 
witli  respect  to  the  left  lower  limb,  it  aiso  still  possessed,  when  the 
neck  of  the  fémur  was  broken,  extensive  and  energetic  movements,, 
modified  merely,  for  some  time  past,  by  motor  incoordination. 

After  thèse  successive  éliminations,  we  are  led  to  admit,  if  I  am 
not  mistaken,  as  an  hypothesis  which  is  at  least  a  very  probable 
one,  that  the  fragility  of  the  bones  has  hère  been  a  conséquence,  in 
some  sort  an  immédiate  one,  of  the  lésion  of  the  nerve-centres.  This 
hypothesis,  being  closely  comiected  with  that  which  I  formerly  pro- 
posed,  in  référence  to  the  détermination  of  the  pathogenic  mode  in 
which  the  arthropaiMes  qf  ataxia  are  produced,  I  believe  I  may 
hold  myself  dispensed  from  hère  re-entering  into  its  discussion,  and 
I  will  confine  myself  to  referring  the  reader  to  the  arguments  which 
I  hâve  severai  times  brought  forward.^ 

This  vice  of  nutrition,  subordinated  to  an  influence  of  the  nervous 
System  which  renders  the  bones  brittle  and  which  enables  us  to 
understand  the  occurrence  of  spontaneous  fractures,  is  also,  I  think, 
one  of  the  principal  éléments  that  concur  in  the  production  of  those 
singular  arthropatJiies  of  which  our  observation  présents  a  most 
remarkable  example.  It  is,  in  fact,  known  from  the  description 
I  hâve  given,  that  the  very  rapid  and  extrême  wearing  away  of 
the  articular  extremities  of  the  bones  is  the  principal  character 
which,  from  an  anatomo-pathological  point  of  view,  distinguishes  the 
arthropathies  of  ataxia  from  common  dry  arthritis. 

It  is  not  vi'ithout  interest  to  notice  that  the  production  of  frac- 
tures, supervening  under  the  influence  of  the  most  trivial  causes,  is 
far  from  being  an  absolutely  rare  fact  in  the  history  of  progressive 
locomotor  ataxia.  I  hâve,  for  my  own  part,  already  met  with  a 
certain  number  of  examples  of  this  kind,  and  quite  recently  Dr. 
Weir  Mitchel  "  called  attention  to  the  fragility  of  the  bones  of  the 
lower  extremities  in  ataxic  patients,  and  to  the  frequency,  in  thèse 
patients,  of  the  fractures  termed  spontaneous.     I  will   add  that 

'  "  Sur  quelques  arthropathies  qui  paraissent  dépendre  d'une  lésion  du 
cerveau  ou  de  la  moelle  épinière,"  in  '  Areh.  de  Phys.,'  t.  1er,  p.  i6i.  See 
also,  idem,  t.  ii,  p.  121,  and  t.  iii,  p.  306;  'Leçons  sur  les  Maladies  du 
Système  Nerveux,'  t.  ii,  p.  54. 

2  "  The  Influence  of  Rest  in  Locomotor  Ataxy."  '  The  American  Journal  of 
Médical  Science,' July,  1873,  113,  116;  and  '  Centralblatt,' p.  720,  5  Oct., 
No.  45,  1873. 


.516  LOCOMOÏOE  AÏAXIA. 

amongst  the  cases  which  hâve  beeu  collected  in  the  différent  writ- 
ings  consecrated  to  the  study  of  this  kind  of  fractures,  there  is  a 
certain  number  in  which  we  maj  perceive — although  they  hâve  not 
been  noticed  by  the  writers — tabetic  symptoms,  and  particularly 
attacks  of  hghtning  pains.  I  will  mention  as  examples,  amongst 
others,  the  cases  No.  32,  and  ^^,  of  the  work  of  Dr.  E.  Gurlt.^ 

Extract  from  the  '  Archives  de  Physiologie  Normale  et  Patho- 
logique/ 1874,  p.  166. 

'  E.  Giirlt,  '  Handbuch  der  Lelire  von  den  Knochenbriicheu,'  ler  Tbeil,  p. 
J47,  'Die  Knochenbrûchigkeit.' 


II. 

ON  THE  TUMEFACTION  OF  THE  MOTOR  NERVE-CELLS  AND 
OF  THE  AXIS-CYLINDERS  OF  THE  NERVE-TUBES  IN 
CERTAIN  CASES  OF  MYELITIS.    Bt  J.  M.  Chaecot. 

(See  Lecture  X,  p.  144.) 

In  tlie  case  of  subacute  myelo-ineningitis,  whicli  he  lias  made  the 
subject  of  a  work  full  of  important  détails,  Dr.  C.  Frommann  lias 
noted  with  care  the  remarkable  tuméfaction  wbicli  is  hère  and  there 
presented  by  the  axis-cylinders  of  the  nerve-tubes  of  the  white 
substance,  not  only  at  the  most  gravely  injured  part  of  the  cord, 
but  also  at  a  great  distance  from  this  focus,  in  spots  which  he 
believes  to  bave  been  affected  in  a  secondary  manner.^  I  hâve 
recently  had  occasion  to  meet  with  this  altération  of  the  axis- 
cylinders,  in  three  cases  of  irritative  lésions  of  the  spinal  cord  ;  the 
observations  which  I  bave  made  in  référence  to  this  subject  will 
allow  me  to  confirm  and  complète,  in  some  respects,  the  description 
given  by  Dr.  Frommann.  I  may  add  that,  in  at  least  one  of  thèse 
cases,  I  remarked  a  tuméfaction  of  the  nerve-cells  of  the  anterior 
cornua  which,  if  I  mistake  not,  lias  not  yet  been  noticed  in  such 
cases  ;  and  which  deserves  to  be  brought  into  connexion  with  the 
same  kind  of  altération  presented  by  the  axis-cylinders. 

Obs.  I. — During  the  siège  of  Paris,  a  Garde  Mobile  was 
wounded,  early  one  morning,  in  a  reconnoissance,  and  was  brought  a 
few  hours  afterwards  to  the  ambulance  of  Arcueil,  completely  para- 
lysed  in  bis  lower  extremities.  He  succumbed  the  same  night, 
about  twenty-four  hours  after  the  accident.  A  bail  had  entered 
near  the  right  shoulder,  and  came  out  on  the  opposite  side,  in  the 
lumbar  région.      At  the  autopsy  the  cord  was  found  completely 

"  '  Untersuchung  ûber  die  normale  and  patholog.  Anatomie  des  Rucken- 
markes.'  Jena,  1864,  pp.  98-99,  and  104-105.  See  also,  in  same  work,  figures 
II  and  12  of  plate  iv. 


318  MYELITIS. 

divided  at  the  upper  part  of  tlie  dorsal  région  ;  a  shred  of  dura 
mater  alone  kept  togetlier  the  two  separate  ends  of  the  spinal  cord. 
A  microscopical  examination  of  two  sections  of  the  cord,  in  the 
fresh  state,  taken  from  the  vicinity  of  the  wound,  to  the  extent  of 
about  two  centimètres,  gave  the  following  results.  There  did  not 
seem  to  exist  any  perceptible  altération  of  the  nerve- éléments, 
either  in  the  white  or  grey  substances  ;  no  granulo-cellular  or  non- 
cellular  bodies  ;  no  isolated  fatty  granulations,  on  the  contrary  the 
rayelocytes  appear  perceptibly  more  voluminous  than  in  the 
normal  state.  Many  of  them  are  enveîoped  in  a  thin  layer  of  pro- 
toplasm,  sometimes  globular,  sometimes  slightly  elongated.  Some 
of  the  rudimentary  cells  contain  two  nuclei.  On  the  capillary 
vessels,  the  walls  of  which  exhibit  no  trace  of  granular  fatty  infiltra- 
tion, the  nuclei  are  more  voluminous,  and  many  show  traces  of  seg- 
mentation. A  study  of  the  sections  liardened  in  chromic  acid,  and 
coloured  by  carminé,  enables  us  to  perceive  altérations  which  had 
escaped  the  eye,  in  the  fresh  state.  We  find,  on  transverse  sections, 
scattered  over  différent  spots  of  the  latéral  and  posterior  columns,  a 
kind  of  rounded  or  oval  islets,  in  the  area  of  which  ail  the  axis 
cylinders  hâve  acquired  relatively  considérable  diameter  (fig.  33, 
a).     Some  measurements  yield  the  following  results  :  axis  cylinder 


Fig.  32. — Trmimcdio  myelitis.    a.  Axis-cylinders  of  abnormal  volume,  mea- 
suriug  o/^,  01 — ojit,  0099.     b.  Normal  axis-cylinders  measuring  o/x,oo33. 

remaining  normal  in  the  affected  spots,  o'oo33"^™-  •  hypertrophied 
axis  cylinder,  from  0*0099™™-  to  o"Oi™™-  The  medullary  sheath  is 
not  developed  in  proportion,  and  it  forms  around  the  hypertrophied 
cylinder  a  thin  circular  layer.  Hère  and  there,  some  nerve-tubes 
•with  voluminous  axis-cylinders  are  met  with,  not  assembled  in 
fascicles  like  the  preceding  ones,  but  scattered  and  isolated  in  the 
midst  of  healthy  nerve-tubes. 

The  reticulum  of  the  neuroglia  is  nowhere  thickened,  but  the 
stellate  connective  cells  are  manifestly  more  voluminous  and  more 
clearly  defined  than  in  the  normal  state  ;  some  of  them  enclose  two 
large  nuclei.     The  connective  cells  do  not  appear  more  voluminous 


TCJMEFAGTION    OF  MOTOR  NERVE-CELLS.  319 

or  more  numerous  in  the  places  wliere  tlie  axis-cylinders  hâve  in- 
creased  in  size,  than  in  the  régions  where  the  nerve-tubes  hâve  pre- 
served  ail  the  characters  of  the  normal  state.  Longitudinal  sections 
enable  us  to  note  that  the  hypertrophied  axis-cylinders  hâve  not  an 
uniform  diameter  throughout  their  whole  length;  far  from  that, 
they  présent  fusiform  swellings  at  intervais,  and  it  is  only  at  thèse 
swollen  places  that  they  acquire  the  colossal  dimensions  already 
referred  to.  In  the  parts  between,  the  diameter  of  the  axis-cylin- 
der  go  scarcely  beyond  the  normal  figure.  The  nerve-cells  of 
the  grey  substance  do  not  appear  to  show  any  perceptible 
altération. 

Obs.  2. — I  owe  the  knowledge  of  the  second  case  to  M.  Joffroy, 
who  h  as  enabled  me  to  verify  the  anatomical  détails  connected  with 
it.  It  relates  to  a  tall  and  robust  man,  aged  36.  After  some 
rather  vague,  premonitory  signs,  not  exceeding  the  four-and-twenty 
hours,  this  man  was  taken,  almost  suddenly,  without  perceptible 
cause,  with  complète  paraplegia,  accompanied  by  loss  of  sensi- 
bility  and  rétention  of  urine.  Six  days  after  the  invasion,  the  urine, 
withdrawn  by  means  of  a  cathéter,  contained  blood.  Seventh  day, 
disappearance  of  reflex  movements  in  the  lower  extremities  ;  forma- 
tion of  bedsore  on  the  left  nates.  Eighth  day,  respiratory  disorder  ; 
fetid  urine.  Electric  contractility  is  preserved  in  the  lower  limbs. 
Thirteenth  day,  chilling  of  extremities.  Patient  succumbed  on 
the  fifteenth  day. 

AuTOPSY. — On  a  level  with  the  sixth  and  seventh  dorsal  vertebrœ, 
the  cord  throughout  its  thickness,  with  the  exception  of  a  small 
extent  of  the  posterior  white  columns,  is  softened,  transformed  into 
a  complète  mash,  enclosing  vessels  gorged  with  blood.  Above  this 
chief  focus  the  ramollissement  ascends  to  the  second  and  third 
dorsal  vertebrœ,  gradually  diminishing  in  extent,  and  limiting  itself 
to  the  central  régions  of  the  cord.  Below,  it  descends,  limited 
likewise  to  thèse  régions,  to  near  the  commencement  of  the  lumbar 
«nlargemeut.  Neither  this  nor  the  brachial  enlargement  appears, 
to  the  naked  eye,  to  hâve  undergone  any  perceptible  altération. 

Mleroscopic  exam'mation  in  fresk  siate. — In  the  softened  spots 
are  found  granular  corpuscles,  with  or  without  nuclei,  and  numerous 
free  myelocytes.     There  are  no  leucocytes. 

On  the  parietes  of  the  vessels,  which  présent  also  a  slight  degree 
of  fatty  infiltration,  the  nuclei  are  more  numerous  than  in  the 
normal  state.     In  some  préparations  deeply  pigmented  nerve-cells 


320 


MYELITIS. 


are  found,  which,  however,  do  not  exhibit  any  other  modification  of 
structure. 


FiG.  33. — a,  a.  Largest  axis-cylinders,  measuring  o/i,oi — OjUjOOpç. 
h.  Normal  axis-cjliuders  measuring  0/^,0033. 

Examination  of  sections  hardened  hy  ckromic  acid  and  coloured  hy 
carminé;  iransverse  sections  ;  dorsal  région. — Ail  the  places  wLich 
were  found  softened  hâve  disintegrated  ;  but,  elsewhere  than  in 
thèse  parts  are  found  scattered,  hère  and  there,  a  large  number  of 
small  lacunes  or  foci  of  disintegration,  sometimes  rounded,  some- 
times  oval;  thèse  foci,  chiefly  occupy  the  latéral  or  posterior 
wliite  columns  ;  but  a  certain  number  of  them  is  also  met  with  in 
the  grey  substance,  and  particularly  in  the  posterior  cornua.  In  the 
vicinity  of  thèse  foci,  as  around  the  parts  disintegrated  by  ramol- 
lissement, the  reticulum  of  the  neuroglia  is  remarkably  thickened, 
but  without  the  adjunction  of  fibrillse  of  cord-formation.  The  knots 
of  the  reticulum  hère  présent  star-shaped  spaces,  enclosing  three  or 
four  and  sometimes  fîve  myelocytes.  Many  alvéolée  are  empty; 
others  contain  healthy  nerve-tubes  ;  most  enclose  axis-cylinders, 
gcnerally  deprived  of  medullary  matter,  the  diameter  of  which 
largely  exceeds  the  normal  standard.  The  largest  of  thèse  axis- 
cylinders  measure  0,045™°'-  j  others  0,035"°^-  ;  others,  again, 
0,025™"'-  only,  the  normal  condition  being  represented  by  the 
figure  0,0035™'°'     Beyond  the  vicinity  of  the  foci  of  disintegra- 


TUMEFACTION  OF  MOTOR  NERVE-CELLS. 


3^1 


tion^  in  the  spots  where  the  connective  reticulum  is  not  thickened, 
tlie  large  axis-cylinders  are  found  sometiines  isolated  and  scattered 
hère  and  tliere,  and  sometimes  united  in  rounded  or  oval  groups. 
They  are  also  seen  either  isolated  or  clustered  in  the  cervical  and 
lumbar  régions  of  the  spinal  cord,  where  the  foci  of  disintegration 
of  the  reticulum-hyperplasia  are  completely  absent. 


Pi  G.  34. — a.  Largest  axis-cylinders. 

An  examination  of  the  longitudinal  sections  shows  that  the  ex- 
traordinary  dimensions,  whicli  hâve  just  been  statedj  correspond  to 
the  fusiform  swellings  of  the  axis-cylinders;  in  the  intervais 
between,  the  cylinder  recovers  almost  its  normal  diameter  (fig.  34) . 
We  can  follow  the  axis-cylinders  with  moniliform  dilatations  on 
successive  sections  representing  a  length  of  from  half  to  one  centi- 
mètre ;  above  and  below,  they  résume  their  normal  character. 

By  means  of  comparative  measurements,  we  appear  to  see  that 
a  considérable  number  of  the  motor  nerve-cells,  in  différent  régions 
of  the  cord,  hâve  increased  in  volume.  However  this  may  be,  this 
cell-swelling  is  much  less  marked  and  less  easily  determined  than  in 
the  following  case. 

TOL,   II.  31 


322  MYELITIS. 

Obs.  III. — This  third  case  was  noted  at  tlie  Hôpital  de  la  Pitié 
hj  M.  Bourneville,  who  left  to  me  tlie  task  of  tlie  liistological 
examination  of  the  spinal  cord.  With  respect  to  tlie  détails  of  the 
clinical  history  and  tlie  macroscopic  anatomy,  I  would  refer  the 
reader  to  the  account  published  by  M.  Bourneville,  in  No.  40 
of  the  'Gazette  Médicale'  for  1871  (yth  Oct.,  p.  451),  as  I  désire 
to  confine  myself  to  some  very  summary  remarks  on  thèse  points. 
The  subject  of  this  observation  is  a  woman,  aged  58,  who  awoke 
one  morning  with  a  rather  sharp  pain  in  the  nape  of  the  neck,  and 
a  contraction  of  the  right  sterno-cleido-mastoid  muscle.  Pive  days 
later  an  incomplète  motor  paralysis,  with  diminished  sensibility  of 
the  left  upper  extremity,  sliowed  itself.  On  the  eiglitli  day  from 
the  appearance  of  the  first  symptoms  death  supervened  suddenly, 
in  conséquence  of  a  fit  of  dyspnœa.  In  this  woman's  case  the  dis- 
ease,  it  would  appear,  had  shown  itself  without  forewarnings.  The 
patient,  however,  stated  that  she  had,  two  years  before,  experienced 
symptoms  very  analogous  to  her  récent  symptoms,  which,  after  a 
very  short  time,  disappeared,  leaving  no  trace. 

AuTOPSY. — Transverse  sections  of  the  spinal  cord,  after  only  a 
few  days'  macération  in  dilute  chromic  acid  show,  in  the  latéral  half 
in  the  cervical  région,  an  oval  blood-clot  focus,  measuring  4  milli- 
mètres in  its  widest  part  (antero-posterior  diameter),  by  3  milli- 
mètres (transverse  diameter),  which  extcnds,  in  height,  from 
about  the  level  of  the  first  cervical  pair  to  that  of  the  seventh. 
This  focus  occupies  the  internai  and  posterior  half  of  the  left 
anterior  cornu  of  grey  substance.  It  is  prolonged  forward, 
in  the  substance  of  the  antero-lateral  column  of  the  same 
side,  following  the  direction  of  the  intra-spinal  course  of  the  an- 
terior roots. 

The  hsemorrhage  is  only  partially  of  récent  date.  On  certain 
points  of  the  focus  it  evidently  belongs  to  a  distant  epocli,  for  hère 
and  there  are  found  pigment-granulations  and  rounded  masses  pre- 
senting  the  appearance  of  a  cell,  and  enclosing  clusters  of  blood- 
corpuscles.  In  addition  we  find,  either  in  the  focus  itself,  or  in  the 
substance  of  the  parts  which  constitute  its  parietes  :  1°,  capillary 
vessels,  presenting  moniliform  dilations  at  intervais,  the  walls  of 
which,  laden  with  very  numerous  nuclei,  présent  hère  and  there 
clusters  of  fatty  granulations  ;  2°,  axis-cylinders  deprived  of  medul- 
lary  matter,  and  much  larger  than  in  the  normal  state  ;  3°,  numerous 
myelocytes,  some  free,  others  enveloped  in  a  small  mass  of  proto- 


TUMEFACTION  OP  MOTOR  NERVE-CELLS.  323 

plasm  ;  4°,  lastly,  débris  of  connective  reticulum,  the  meshes  of 
whicli,  apparently  fibroid,  are  remarkablj  thickened. 

After  complète  hardening  of  the  cord  an  examination  of  trans- 
verse sections  shows,  besides,  what  foUows  :  on  points  of  the  white 
substance^  very  distinct  from  the  walls  of  the  blood-clot  focus — in 
the  most  posterior  part  of  the  posterior  columns,  for  instance — are 
found  irregular  spots  where  the  axis-cylinders,  surrounded  solely 
by  a  layer  of  meduUary  matter,  hâve  generally  acquired  enormous 
dimensions  of  from  0*036™"*-  to  o-oiS"^"*-  in  transverse  diameter. 
In  the  intervais  between  thèse  nerve-tubes  with  swollen  axis-cylin- 
ders  the  meshes  of  the  reticulum  are  sometimes  thickened  ;  generally, 
however,  they  are  no  thicker  than  in  the  normal  state. 

But  what  appears  most  striking  in  this  examination  are  the  truly 
colossal  dimensions  which,  in  the  left  anterior  cornu  of  grey  matter, 
in  the  vicinity  of  the  clot-focus,  are  exhibited  by  the  multipolar 
nerve-cells.  Thus,  whilst  the  largest  cells  of  the  right  cornu 
measure,  on  an  average,  0*0495"™-  in  their  greatest  diameter,  those 
of  the  right  cornu  may  reach  o'oSzs"^'"-.  The  least  voluminous  of 
the  latter  are  still  0*056"*™-  in  diameter,  Besides,  the  tumefied  cells 
are  not  only  more  voluminous  than  in  the  normal  state  ;  they  are 
also  manifestly  deformed.  They  hâve  lost  their  elongated  shape, 
and  become  globular;  they  look  as  if  excessively  distended,  and 
their  parietes  appear  bossed.  Nor  do  the  cell  prolongations  now 
présent  their  nsual  slenderness  ;  they  are  thickened  and  tortuous. 
The  substance  which  forms  the  body  of  the  cells,  thus  altered,  becomes 
strongly  coloured  with  carminé;  it  is  finely  granular,  slightly 
opaline,  and,  moreover,  a  little  opaque,  for  the  glance  with  difficulty 
pénétrâtes  to  the  pigmentary  mass  and  to  the  nucleus.  The  lattir, 
however,  and  the  nucleus  also,  hâve  always  appeared  to  présent  the 
cliaracters  of  the  physiological  condition.  I  was  fortunate  enough 
to  make  Dr.  Lockhart  Clarke,  during  his  last  visit  to  Paris,  a 
witness  to  ail  the  peculiarities  which  hâve  just  been  described. 

The  longitudinal  sections  show  the  moniliform  aspect  of  most 
of  the  swollen  axis-cylinders,  ah'eady  mentioned  in  the  first  two  ob- 
servations. But  one  fact  peculiar  to  the  third  case  is  that  a  certain 
number  of  thèse  voluminous  cylinders  préserve  their  abnormal 
dimensions  uniformly  throughout  a  great  length,  without  traces  of 
dilatation  or  narrowing.  A  final  point  which  deserves  particular 
notice  is  this,  that  in  this  same  case  a  first  examination  in  the  fresh 
state  enabled  us  to  recognise  the  tuméfaction  of  the  axis-cylinders  ; 


324  MYELITIS. 

so  tliat  this  cannot  possibly  be  an  artificial  product,  an  accidentai 
resuit  nf  the  method  of  préparation. 

I  ain  inclined  to  believe  that  tlie  tuméfaction  of  the  axis-cjlinders 
described  in  thèse  observations,  and  that  of  the  nerve-cells  likewise, 
ought  not  to  be  considered  merelj  as  curiosities  of  pathological 
anatomj.  On  the  contrary,  it  appears  very  probable  that  thèse 
altérations  will  be  met  with  again  in  a  good  number  of  cases  of  acute 
or  subacute  myelitis  (vrhere  without  any  doubt  they  play  an  inte- 
resting  part),  M'iien  they  shall  hâve  sufficiently  attracted  the 
attention  of  observers,  and  when,  on  the  other  hand,  our  means  of  ' 
anatomical  research  shall  hâve  been  still  further  improved.  As 
regards  the  swelling  of  the  axis-cylinders,  at  the  moment  of  con- 
cluding  the  présent  note,  I  find  it  mentioned,  once  again,  in  an  ob- 
servation which  forms  part  of  an  interesting  memoir  that  lias  just 
been  published  at  Leipzig  by  Mr.  W.  Miiller.  This  case,  like  the 
first  one  of  those  I  hâve  reported,  relates  to  a  traumatic  lésion  of 
the  spinal  cord.  The  brachial  enlargement,  espccially,  had  been 
injured,  and  at  the  autopsy  it  was  found  softened  ;  death  super- 
vened  thirteen  days  after  the  accident.  The  swollen  and  varicose 
axis-cylinders  were  met  with,  not  only  in  the  vicinity  of  the  focus 
of  ramollissement,  but  also  much  beneath  this  focus  in  the  latéral 
columns  {consécutive  descending  myelitis),  throughout  nearly  the 
whole  length  of  the  dorsal  région.^  It  is  known,  from  the  histolo- 
gical  researches  of  H.  l'rommann,-  and  from  my  own,^  that,  inmost 
forms  of  sclerosis,  and  particularly  in  disseminated  sclerosis,  at  a 
certain  period  of  the  altération,  a  very  perceptible  augmentation  of 
diameter  is  observed  in  a  certain  number  of  axis-cylinders,  besides 
the  fibrillary  metamorphosis  of  the  neurogha  reticulum  ;  indeed,  the 
•tuméfaction,  in  the  latter  case,  is  uniformly  spread  over  a  great 
length  of  the  cylinder,  and  not  simply  localised  in  certain  spots,  as 
occurs  in  acute  myelitis.  However  tins  may  be,  the  altération  in 
question  appears  to  be,  it  is  clear,  common  with  some  variations  to 
primary,  acute,  subacate,  and  chronic  forms  of  inflammation  of  the 
spinal  cord. 

What  is  the  signification  of  this  altération?     If  I  do  not  mis- 

1  'Beitrage  zur  Patholog.  Anatom.  und  Physiol.  der  menschlichen  Riicken- 
marks.'    Leipzig,  1871,  pp.  n— 13. 
-  '  Untersuchungen/  &c.,  2  Tlieil,  Jena,  1867. 
•Histologie  de  la  Sclérose  eu  plaques,'  Paris,  1869,  pp.  11 — 13. 


TUMEFACTION  OP  AXIS-CYLINDEES.  325 

take,  it  ought  to  be  approximated  to  the  swelling  frequentlj 
preseuted  by  various  auatomical  éléments,  glandular  epithelial  cells, 
and  cartilage  capsules,  for  instance,  under  the  influence  of  certain 
irritations.  If  it  be  thus,  the  reader  may  convince  himself  by 
referring  to  the  observations  contained  in  this  note,  that  the  tumé- 
faction of  axis-cylinders  may,  in  certain  cases,  be  the  first  anatomi- 
cal  expression  of  the  inflammation  of  the  spinal  cord.  It  may,  in 
fact,  be  fouud  existing  alone,  independent  of  every  appréciable 
concomitant  altération  of  the  medullary  cylinder,  the  neuroglia- 
reticulum,  and  the  capillary  vessels. 

From  this  point  of  view,  our  first  observation  especially  is  most 
instructive.      It   also   shows   with  what   rapidity    irritation   may 
modify,  in  the  cord,  the  structures  of  the  nerve-elements,  even  in 
l^arts  comparatively  very  distant  from  the  primary  seat  of  mischief. 
The  latter  fact,  that  is,  the  propagation  of  irritation  to  a  distance 
is  equally  well  illustrated  in  our  second  observation  ;  as  also  in  the 
cases  of  MM,  Frommann  and  Millier,  where,  at  a  great  distance 
from  the  principal  focus,  we  see  the  axis-cylinders  swollen,  hère  and 
there,  either  along  the  course  of  the  posterior  columns,  or  in  that  of 
the  latéral  fascicles.     Everything  tends  besides  to  show  that  the 
irritation  of   the  nerve-tubes  and,  more  explicitly,  of  their  axis- 
cylinder  is,  in  acute  or   subacute  myelitis,  sometimes  the  initial 
primordial  fact  {parench/viatous  myelitis)  -,  sometimes,  on  the  con- 
trary,  a  deuteropathic  phenomenon,  consécutive  on  inflammation  of 
the  connective  reticulum  {interstitial  myelitis).     Hence,  we  hâve 
reason  to  apply  to  acute   or    subacute  myelitis    the  fundamental 
distinction  proposed  by  M.  Vulpian,^  in  référence  to  spinal  scléroses. 
The  morbid  process,  the  most  salient  features  of  which  hâve  just 
been  recalled  may,  if  it  be  not  hindered  in  its  development,  issue  in 
the  disintegration,  and  finally  in  the  complète  destruction  of  the 
axis-cylinder.     It  is  not,  in  fact,  rare  to  meet,  in  cases  of  some 
standing,  with  a  certain  number  of  swollen  axis-cylinders,  which, 
when  examined  in  longitudinal  sections  of  the  cord,  appear  to  be 
unequal,  humped  on  the  surface,  and  furrowed  across  with  more 
or  less  deep  slits.     At  the  most  advanced  stage,  owing  to  the  aug- 
mentation of  thèse  slits,  the  swollen  part  of  the  cylinders  may  be 
divided  into  several  irregularly  globular  and  independent  masses. 
This  mode  of  altération  was  very  marked  in  our  second   and  third 
observations  ;  it  has  been  perfectly  described  in  the  cases  of  MM. 
)  '  Archives  de  Physiologie,'  t.  ii,  p.  289. 


326  MYELITIS. 

Frommann  and  W.  Miiller.^  When  matters  are  at  this  point,  the 
axis-cylinders  hâve  been  already  long  deprived  of  their  medullary 
covering.  It  is  scarcely  doubtful  that  the  swollen  nerve-cells  may, 
also,  expérience  a  consécutive  atrophy,  corresponding  to  this  disin- 
tegration  of  the  axis-cyhnders.  I  shall  confine  myself,  at  présent, 
to  thèse  remarks,  which  I  hope  soon  to  résume  and  develop  in  a 
gênerai  study  on  the  pathologie  histology  of  acute  myelitis  ;  but, 
I  do  not  wish  to  conclude  this  note  without  pointing  out  that  the 
tuméfaction  of  the  axis-cylinders  does  not  exclusively  pertain  to  the 
nerve-tubes  of  the  spinal  cord.  I  hâve  myself  observed  it  several 
times  in  parts  of  the  brain  aff'ected  with  ansemia,  but  not  yet 
softened,  in  conséquence  of  the  oblitération  of  an  artery  of  the 
brain  by  thrombus. 

Moniliform  tuméfaction  of  the  axis-cylinders  lias  besides,  been 
long  observed  in  the  retina,  by  MM.  Zenker,^  Virchow,^  H., 
Millier,*  Schweigger,^  and  Nagel,^  in  the  brain  (yellow  ramollis- 
sement of  the  cortical  layer,  and  congénital  interstitial  encephalitis) 
by  H.  Virchow.7  Lastly,  and  more  recently,  Dr.  H.  Hadlich  ^  has 
noted  the  varicose  swelling  of  the  axis-prolongations  {Hauptaxen- 
ci/linderforsatz),  of  the  great  nerve  cells  of  the  cortical  layer  of  the 
cerebellum  in  a  patient  affected  with  cerebellar  haemorrhage. 

(Extract  from  the  '  Archives  de  Physiologie  Normale  et  Patholo- 
gique,' 187 1—1872,  p.  93.) 

*  'Archives  de  Physiologie,'  t.  11,  p.  289. 

-  'Archiv  fur  Ophth.,'  Bd.  il,  S.  137. 

^  '  Virchow's  Archiv,'  Bd.  x,  S.  175, 

"  'Archiv  fur  Ophthal.,'  Bd.  iv,  2,  S.  i. 

^  Ibid.,  Bd.  vi,  2,  S.  294. 

«  Ibid.,  Bd.  vi,  i,  S.  191. 

'  '  Virchow's  Archiv,'  Bd.  x,  S.  178,  aud  Bd.  xliv,  S.  475. 

«  Ibid.,  Bd.  vi,  4°  fig.  S.  218,  1869. 


III. 

NOTE  ON  A  CASE  OP  SPINAL  PROTOPATHIC  PROGRESSIVE 
MUSCULAR  ATROPHY  (DUCHENNE-ARAN  TYPE).  By  J.  M- 
Charcot. 

(See  Lecture  XI,  p.  163). 

The  principal  clinical  characters  which  allow  us  to  diagnose,  pro- 
topathic  muscular  atrophy,  during  life,  will  be  found  assembled  in 
the  following  observation,  wliicli  is  a  remakable  example  of  this 
form  of  chronic  amyotrophy.  Here^  in  fact,  the  diagnosis,  which 
anatomical  examination  afterwards  coufirmed,  was  made  during  the 
patient' s  lifetime  : 

Laure  W —  was  admitted  to  the  Salpêtrière,  March  içth.  She 
was  affected  with  progressive  muscular  atrophy,  the  first  symptom& 
of  which  she  had  experienced  in  1862,  at  the  âge  of  about  thirty- 
seven. 

The  jjatient's  history  gave  only  négative  results  on  inquiry  ;  none 
of  the  members  of  her  family  had  been  affected  with  muscular 
atrophy,  nor  had  she  herself  ever  had  any  disease,  save  the  erup- 
tive  fevers  of  childhood;  finally,  she  had  been  a  sempstress  ail  her 
life,  and  lias  never  experienced  privations,  nor  inhabited  an  un- 
healthy  dwelling. 

A  certain  progressive  weakness,  not  accompanied  by  any  sensory 
disturbance,  marked  the  invasion  of  the  symptoms  in  the  upper 
extreraities.  Soon  after  atrophy  attacked  the  muscles  of  the  left 
hand  ;  then,  six  months  later,  the  right  was  attacked,  in  its  turn. 
Dating  from  this  period,  the  disease  pursued  its  career  in  a  regular 
and  slow  manner,  occupying  one  after  another,  from  below  upward, 
the  several  segments  of  the  upper  extremities,  but  proceeding  much 
more  rapidly  in  the  right  than  in  the  left  side.  It  should,  how- 
ever,  be  remarked  that,  for  about  eight  years,  the  lower  extremi- 
ties had  been  the  seat  of  singular  phenomena.  Real  paroxysms  of 
pain  used  to  occur,  accompanied  by  violent  muscular  jerks.  Thèse 
fits  corne  on,  by  préférence,  whilst  the  patient  was  in  bed;  but 
they  might  also  take  place  whilst  she  was  walking,  and  they  were 
violent  enough  to  compel  her  to  perform  odd  movements  (curtseys^. 


S28  PROTOPATHIC  MUSCULAR  ATROPHY- 

bows,  &c.).i  They  were  calmed  by  the  application  of  cold  water 
to  the  lower  extreinities. 

Présent  state,  IMay,  1869,  seven  years  after  the  invasion  of  the 
disease. — The  atrophy  bas  invaded  both  hands,  both  amis,  both 
shoulders,  especially  the  right,  as  h'kewise  the  muscles  of  the  supra- 
and  sub-spinous  fossœ.  No  librillary  movements  are  noticed  either 
in  the  forearms,  nor  in  the  arms,  but  they  hâve  a  very  distinct  exist- 
ence in  the  right  shoulder,  where  they  are  determined  by  the 
slightest  shock  ;  they  are  likewise  observed  in  almost  the  whole  of 
the  upper  part  of  the  back.  The  lower  limbs  are  perfectly  free  ; 
the  patient  walks  about  the  whole  day  long.  Nothing  noticeable 
in  face,  tongue  or  larynx.  From  time  to  time  slight  lits  of  dys- 
pnœa,  but  not  of  an  urgently  threatening  character.  About  a  year 
ago,  the  patient  experienced  pains  in  the  neck,  on  the  side  of  the 
vertébral  column,  in  the  latéral  muscle-masses.  To-day,  thèse 
pains  hâve  disappeared,  but  similar  ones  hâve  conie  on  in  the  left 
side  of  the  neck.  In  addition,  for  some  time  past,  the  patient 
experienced  formications  and  numbness  in  both  arms. 

1872. — The  patient  was,  for  six  months,  put  under  treatment  by 
continued  (descending)  currents,  but  if  there  were  any  improve- 
ment,  it  did  not  last.  The  atrophy  whilst  making  progress  in  the 
upper  extremities  and  in  the  trunk,  had  not  perceptibly  invaded 
the  lower  limbs,  the  functions  of  which  were  freely  carried  on. 
Still,  the  patient  always  complained  of  the  existence  of  pains, 
coming  on  by  fits  and  starts,  in  the  neck,  the  back,  and  the  lower 
extremities  ;  and  it  was  directly  determined  that  pressure  on  the 
spinous  apophyses  was  painful  ail  along  the  vertébral  column. 

From  1872  to  1875  the  state  of  the  patient  remained  much  the 
same.  She  was  several  times  shown  to  those  who  assisted  at  the 
lectures  given  in  the  Salpêtrière,  as  an  example  of  protopathic 
spinal  muscular  atrophy.  This  diagnosis  was  based  upon  the  fol- 
lowing  chief  characters  :  in  spite  of  the  considérable  réduction  in 
volume  which  the  muscular  mass  of  the  upper  extremities  had  un- 
dergone,  thèse  were  not  in  reality  affected  by  true  paralysis  ;  certain 
partial  movements  were  possible,  particularly  the  movement  of  élé- 
vation by  the  shoulder-musclcs.  Thanks  to  thèse  movements,  the 
patient  could  yet  to  some  extent  make  use  of  lier  hands.  By  means 
of  some  manœuvring  she  could  still  manageto  turn  the  handle  of  a 
lock ,  open  a  drawer,  lift  a  chair,  or  at  least  drag  it  where  she  pleased. 
^  lu  coujunction  witli  M.  Gombault. 


COURSE  OF  THE  DISEASE.  329 

Generally  speaking,  lier  upper  extremities  were  flaccid,  usually 
hanging  down  by  her  sides,  free  from  articulai  stiffness.  The 
fingers,  however,  were  flexed  ou  the  palm  of  the  hand,  without  its 
being  actually  possible  to  exteud  them. 

The  lower  extremities  were  quite  free;  their  muscular  masses 
were  volumiuous  ;  the  patient  walked  easily,  and  eveu  weut  rather 
long  distances  outside  the  hospital,  without  great  fatigue. 

1875. — The  atrophy  of  the  muscles  is  extremely  marked  in  the 
upper  parts  of  the  body.  It  bears^  almost  equally,  on  both  upper 
extremities.  The  deltoids^  the  pectoral  muscles^  are  almost  com- 
pletely  destroyed;  the  intercostal  spaces  are  deeply  hollowed  out; 
the  same  thing  is  seen  in  the  supra-  and  sub-spinous  régions  ; 
throughout  ail  the  upper  portion  of  the  body  the  skeleton  shows 
itself,  and  seems  merely  covered  by  the  skin.  The  head,  no  longer 
upheld  by  the  muscles  of  the  neck,  falls  forward,  and  remains 
habitually  flexed  upon  the  chest.  Prom  this  attitude  a  certain 
difïiculty  of  déglutition  results,  which  is  probably  altogether  me- 
chanical;  the  patient  can  only  swallow  witli  great  trouble  whilst 
sitting,  and  consequently  she  lias  to  eat  standing.  The  dorso- 
lumbar  column  is  greatly  curved  in,  and  the  arms,  which  are  always 
flaccid,  hang  usually  beliind  the  chest. 

Still  the  lower  extremities  are  voluminous^  and  walking  is  always 
easy.  The  difïiculty  of  breathing  has  become  very  great;  the 
utterance  is  broken,  panting,  and  the  voice  somewhat  liusky 
(voilée)  ;  however,  the  articulation  of  souiids  is  perfectly  distinct  ; 
the  tongue  has  preserved  its  original  volume  and  the  freedoin  of  ail 
its  movements.  The  vocal  troubles  should,  therefore,  be  attributed 
to  the  difficulty  of  breathing,  which  is  remarkably  increased  by  efforts 
and  by  walking,  the  patient  cannot  go  up  a  flight  of  stairs  without 
getting  palpitations  at  once.  lier  puise  was  not  counted  in  1875; 
in  1873,  it  was  calm  and  regular.  The  cutaneous  sens'ibïlity  is 
normal,  pressure  on  the  muscular  masses  does  not  cause  pain,  the 
intellect  is  perfectly  preserved. 

During  the  last  two  months,  the  gênerai  debility  makes  rapid 
progress;  the  appetite  is  completely  lost,  vomitings  supervene, 
copions  leucorrhœa,  and,  lastly,  œdema  of  the  feet  and  hands.  At 
the  same  time,  the  respiration  grows  more  and  more  embarrassed. 
However,  in  spite  of  this  state  of  extrême  weakness,  the  patient 
continues  to  walk  a  little,  and  on  the  day  before  her  death  she  was 
still  able  to  go  to  the  consulting-room  of  the  infirmary. 


330  PEOTOPATHIC  MUSODLAR  ATROPHY. 

On  tlie  i(Sth  of  May,  it  was  impossible  for  lier  to  quit  her 
dormitory  to  go  to  the  chapel,  as  she  was  accustomed.  During 
tlie  niglit,  she  was  seen  to  leave  her  bed  in  order  to  go  and  sit  in 
her  arm-chair  ;  as  she  remained  there  for  a  considérable  time 
without  stirring,  her  neighbours  called  her  ;  they  came  to  her, 
found  her  motionless^  extremely  pale,  having  nearly  lost  conscious- 
ness  altogcther.  She  was  put  back  in  her  bed,  where  she  soon 
died. 

AuTOPSY,  May  19.  Nervous  System. — The  brain  and  cerebellum 
are  not  the  seat  of  any  lésion  ;  neither  are  the  protuberantia  or 
bulbus  affected.  The  bulbar  nerve-roots  are  normal  in  size,  and 
hâve  their  usual  white  colour. 

Spinal  cord. — The  anterior  roots  are  reddish,  transparent,  mani- 
festly  wasted.  Their  colour  contrasts  with  the  fair  white  hue  of 
the  posterior  roots,  which  appear  healthy.  This  state  of  the 
anterior  roots  is  only  met  with  in  the  cervical  and  dorsal  régions. 
It  ceases  almost  entirely  at  the  first  lumbar  pair.  The  cord  itself 
does  not  exhibit  any  grey  coloration  on  its  surface,  its  tissue  is 
neither  softened  nor  indurated,  there  is  no  manifest  thickening  of 
the  méninges.  On  a  transverse  sectioUj  taken  in  the  cervical 
région,  we  remark  the  gelatinous  aspect  of  the  anterior  cornua, 
and  the  total  absence  of  ail  grey  tint  on  a  level  with  the  latéral 
columns. 

Muscles.— hi  a  gênerai  manner,  the  affected  muscles  are  dis- 
coloured  ;  they  hâve  assumed  the  yellow  dead-leaf  tint,  and  hâve 
undergone  a  more  or  less  considérable  réduction  in  size  ;  nowhere 
do  we  observe  remarkable  adipose  substitution.  They  are  not, 
however,  ail  affected  to  the  same  extent,  and  some  may  even  be  met 
with  which,  like  the  trapezius,  are  only  partially  atrophied. 

Upper  right  limb. — The  deltoid  is  yellow,  and  considerably 
thinned.  In  the  arm,  the  triceps  alone  has  retained  a  size  and 
colour  which  approach  to  the  normal  state  ;  ail  the  other  muscles  of 
this  région  are  more  or  less  wasted  and  discoloured.  They  are, 
however,  less  thoroughly  affected  than  the  muscles  of  the  forearm, 
which,  for  the  most  part,  are  reduced  to  thin  membranes;  the 
flexor  carpi  ulnaris  and  the  palmaris  longus  hâve  almost  completely 
disappeared  ;  the  flexor  carpi  radialis  and  the  pronator  radii  teres 
are  a  little  less  wasted.  In  the  posterior  région,  ail  the  muscles 
are  deeply  affected,  with  the  exception  of  the  extensor  pollicis 
longus,  which  is  still  red  and  rather  larger.      In  the  hand,   the 


HISTOLOGICAL  EXAMINATION.  331 

lumbricales  liave  alone  preserved  their  red  hue,  and  a  certain  degree 
of  prominence. 

Loiver  r'ight  l'imh. — Hère  ail  the  muscles  retain  a  still  considér- 
able volume  and  their  normal  colour;  none  of  them  is  manifestly 
wasted.  The  sacro-lmnbar  mass  is  perfectly  preserved;  at  the  nape 
of  the  neck,  on  the  contrary,  most  of  the  muscles  are  wasted  and 
discoloured.  The  latissimus  dorsi  and  the  whole  of  the  posterior 
part  of  the  trapezius  hâve  undergone  extrême  atrophy.  The  cla- 
vicular  fascicle  of  the  latter  muscle  is,  on  the  contrary,  red  and 
voluminous.  The  muscles  of  the  supra-  and  sub-spinous  fossse  are 
wasted.  The  pectoral  and  intercostal  muscles,  and  the  muscles  of 
the  anterior  part  of  the  abdomen  hâve  undergone  extrême  atrophy. 
The  intercostal  space  is  absolutely  translucid.  The  diaphragm  has 
thinned  greatly  ;  on  raising  the  peritoneum,  we  can  see  numerous 
yellow  streaks,  corresponding  to  bundles  of  diseased  fibres,  alter- 
nating  with  less  numerous  red  fibres.  The  sterno-mastoid  muscles 
appear  almost  healthy,  as  well  as  the  supra-  and  sub-hyoid  muscles. 
The  muscles  of  the  pharynx  are  of  a  fine  red,  and  remarkable  for 
their  thickness  ;  those  of  the  larynx  appear  equally  normal.  The 
longue  has  its  normal  volume^  its  différent  muscular  layers  are  red, 
there  is  no  trace  of  fatty  substitution.  The  différent  viscera 
appeared  healthy. 

Histological  examination.  Cervical  région. — The  dissection  of 
small  fragments  of  substance,  taken  on  a  level  with  the  anterior 
cornua  allows  us  to  perceive  the  existence  of  altérations  whicli  bear 
alike  on  the  vascular  parietes,  on  the  interstitial  éléments,  and  on 
the  nerve-cells. 

State  of  the  vessels. — Generally  speaking,  the  condition  hère  was 
that  of  a  chronic  irritative  process,  determining  the  formation  of 
new  éléments,  and  issuing  in  a  sometimes  considérable  thickening 
of  the  vascular  parietes.  The  différent  phases  of  this  hyperplasic  pro- 
cess may  be  followed  easily  enough  on  the  walls  of  the  capillaries. 

At  an  early  stage,  at  intervais  over  the  external  surface  of  thèse 
walls  large  nuclei  are  met  with,  taking  a  deep  tint  with  carminé, 
and  surrounded  by  a  rather  abuudant  granular  protoplasm.  Thèse 
éléments  make  a  noticeable  prominence  on  the  surface  of  the  vessel, 
and  are  readily  distinguished  from  the  nuclei  of  the  epithelium, 
which  are  paler,  and,  in  certain  positions,  prominent,  on  the  con- 
trary, towards  the  concave  side.  Further  on,  prolifération  is  pro- 
duced,  then  we  find  sometimes  three  or  four  nuclei  united  together 


332  PROTOPATHIO  MUSGULAK  ATliOPHY. 

by  a  common  mass  of  protoplasin  ;  sometimes  clusters  of  young 
cells  more  completely  developed  (PI.  VII,  fig.  6,  a,  h). 

It  is  probable  tliat  thèse  éléments  are  susceptible  of  undergoing, 
afterwards,  modifications  which  bring  tliem  to  a  more  comj)lete 
organisation  ;  for  in  some  spots,  we  fiud  true  fusiform  bodies,  fiir- 
nislied  witli  very  long  prolongations  and  forming  a  sort  of  incom- 
plète adventitious  coat  to  the  capillary.  The  proper  wall  of  most 
of  thèse  capillaries  has  undergone  a  very  noticeable  thickening. 
As  to  the  endothelium,  it  does  not  appear  to  be  manifestly  modi- 
fied.  In  spite  of  an  attentive  examination  it  has  been  impossible 
for  us  to  distinctly  ascertain  the  fact  of  the  neoformation  of 
capillaries. 

In  vessels  provided  with  several  coats,  the  phenomena  which  we 
liave  just  described  are  also  produced.  Hère,  agaiu,  it  is  the  external 
parts  of  the  wall  which  were  the  almost  exclusive  seat  of  the  morbid 
process  ;  the  lymphatic  sheath  is  thickened,  its  cavity  no  longer 
exists,  it  adhères  closely  to  the  surface  of  the  vessel.  Ilowever, 
the  aspect  exhibited  by  thèse  parts  varies,  very  probably,  with  the 
âge  of  the  lésion;  sometimes  they  are  charged  with  abundant 
cellular  éléments,  sometimes,  on  the  contrary,  it  is  the  fibrous  élé- 
ment which  prédominâtes.  Lastly^  we  sliould  notice,  in  thèse 
same  spots,  the  habituai  présence  of  leucocytes,  occasionally  so 
numerous  as  to  almost  completely  mask  ail  other  histological 
détails  (PI.  VII,  fig.  7).  Hère  again  the  internai  membrane 
and  the  endothelium  in  particular,  appear  to  be  respected.  As 
to  the  middle  coat,  it  is  evidently  altered  in  certain  points.  Its 
muscular  fibres  become  granular,  and  a  number  of  thcm  hâve 
disappeared. 

The  altération  of  the  neuroglïa  is  betrayed  by  the  présence  of 
some  granular  bodies,  by  a  very  considérable  number  of  cellular 
éléments,  some  of  which  présent  the  characters  of  multiplication 
by  scission  (hour-glass  nucleus),  and  finally  by  the  unusual  abun- 
dance  of  fibrillary  tissue. 

As  to  the  nerve-cells,  they  hâve  largely  disappeared.  We  may 
go  over  entire  préparations  without  finding  a  single  cell  of  any  con- 
sidérable size.  A  strong  magnifying  power  must  be  employed,  in 
order  to  distinguish  a  number  of  thcm.  The  smallest  that  can 
still  be  discerned  are  coustituted  by  a  voluminous  nucleus,  readily 
coloured  by  carminé,  provided  with  a  large  nuclcolus  and  sur- 
rouuded  by  a  small  rounded  or  oval  zone  of  ycUow  pigment  (PI. 


LESIONS  or  MOTOE  NERVE-CELLS.  33o 

VII,  fig.  4,  a,  a).  Some  others,  less  reduced  in  size,  hâve  pre- 
served  vestiges  of  tlieir  prolongations  in  tlie  form  of  angles  ;  their 
uucleus  is  nearly  normal,  like  that  of  tliose  already  mentioned,  and 
their  protoplasma  is  quite  infiltrated  with  pigmentary  granulations 
(PI.  VII,  fig.  4,  1j).  In  a  word,  atrophy  of  thc  protoplasm,  loss 
of  the  prolongations,  comparative  increase  of  the  yellovv  pigment, 
long  persistence  of  the  normal  characters  of  the  nucleus  and 
nucleolus, — such  are  the  characters  of  the  process  which  hère  pré- 
sides over  the  destruction  of  the  nerve-cells. 

In  the  lumbar  région  the  substance  of  the  anterior  cornua  is 
comparatively  healthy;  the  nerve-cells  are  abundant,  most  of 
them  appear  healthy,  and  merely  a  little  more  pigmented  than 
usual.  However,  some  of  them,  but,  indeed,  only  a  few,  are  found 
to  be  as  completely  wasted  as  those  of  the  cervical  région.  The 
walls  of  the  vessels  are  not  absolutely  healthy,  in  the  large  vessels 
especially  thcy  are  maiiifestly  thickened. 

Portions  of  the  white  substance,  taken  from  the  centre  of  the 
latéral  columns,  in  the  cervical  and  lumbar  régions,  contained  in 
the  fresh  state  no  granular  body.  Some  fragments  of  the  nucleus 
of  the  hypoglossus,  examined  by  the  method  of  dissociation, 
exhibited  the  perfect  integrity  of  ail  the  éléments  which  com- 
posed  it. 

Examination  of  sections  after  harclening  in  chromic  acid. — The 
grey  substance  of  the  anterior  cornua  is  gravely  altered  in  the 
cervical  and  dorsal  régions.  The  maximum  of  the  altérations 
occupies  the  lower  part  of  the  cervical  enlargement.  Above  and 
below  this  spot  they  diminish  as  they  go.  At  this  level,  we  note 
the  almost  complète  disappearance  of  nerve-cells,  and  of  most  of 
the  medullated  tubes,  which,  in  the  normal  state,  traverse  the  area 
of  the  anterior  cornu,  in  every  direction.  It  results  from  this, 
that  the  grey  substance  takes  with  carminé  a  much  deeper  tint 
than  in  the  normal  state.  In  spite  of  the  disappearance  of  the 
nerve  éléments,  the  dimensions  of  the  anterior  cornua  hâve  not 
noticeably  diminished.  This  must  be  largely  attributed  to  the 
truly  enormous  development  taken  by  the  capillary  system  of  the 
région. 

In  the  upper  parts  of  the  cervical  région,  the  vascularisation 
diminishes,  medullated  tubes  appear  anew,  hère  and  there  some 
recognisable  nerve-cells  are  remarked  ;  some  of  them,  two  or  three 
at  most,  in  each  préparation,  are  even  almost  normal  in  their 


334  PROTOPATHIO  MUSOULAR  ATROPHY. 

dimensions.  In  tlie  dorsal  région,  the  lésion  becomes  equally 
lessened  as  it  approaches  the  lumbar  région. 

On  a  levcl  witli  the  lumbar  enlargement,  the  grey  substance  has 
almost  completely  resumed  the  characters  of  the  normal  state. 
The  nerve-cells  are  numerous,  provided  with  prolongations,  col- 
lected  in  distinct  clusters,  nearly  ail  contain  a  considérable  mass  of 
pigment. 

At  long  intervais  an  atrophied  cell  is  met  with.  The  large 
vessels  hâve  their  walls  thickened,  but  much  less  than  in  the  cervical 
région.  This  state  of  the  vessels  is  again  met  with  throughout  the 
whole  extent  of  the  posterior  cornua  which  are  more  vascularised 
than  is  usual. 

Throughout  the  entire  cord,  the  latéral  columns  properly  so- 
called,  TiircFs  columns,  and  the  posterior  fascicles  are  untouched. 
But  the  great  vascular  tracts  which  traverse  them  are  a  little  larger 
than  usual,  and  the  vessels  which  they  contain  hâve  their  walls 
thickened. 

As  to  the  anterior  radicular  zone,  it  is  attacked  with  sclerosis  in 
the  cervical  and  dorsal  régions.  The  extent  of  this  sclerosis  is 
manifestly  in  relation  with  the  intensity  of  the  lésion  which  occupies 
the  anterior  cornu,  it  augments,  diminishes,  ends  simultaneously 
with  it.  It  is  also  largely  dépendent  on  the  lésion  of  the  anterior 
roots  in  their  intra-spinal  course.  Thus,  in  the  inferior  part  of  the 
cervical  enlargement,  it  forms  around  the  anterior  cornu  an  almost 
complète  belt,  which  sends  towards  the  circumference  broad  pro- 
cesses, while  in  the  dorsal  région  it  is  limited  to  the  cornu  of  the 
radicular  fascicles  and  cortical  zone. 

In  addition,  at  the  point  of  émergence  of  tlie  anterior  roots,  the 
pia  mater  is  thickened  and  manifestly  inflamed.  This  infiammatory 
state  of  the  pia  mater  is  met  with  again,  though  less  marked,  ou 
every  point  of  the  periphery  of  the  cord.  It  has  reacted  upon  the 
immediately  subjacent  layer,  and  the  resuit  is  a  thin  zone  of  cortical 
sclerosis,  the  maximum  thickness  of  which  is  at  the  level  of  the 
anterior  radicular  fascicles,  diminishing  over  the  latéral  columns,  to 
disappear  altogether  on  a  level  with  the  posterior  columns.  In  the 
lumbar  région,  thèse  varions  lésions  disappear,  the  cortical  layer 
îind  the  anterior  radicular  zone  are  in  a  state  of  perfect  health. 

Bulbus  rachidiais,  section  taken  from  the  middle  of  the  olivary 
hodïes. — The  anterior  pyrmiids  are  healthy.  The  hypoglo&ms  nûcletts 
is  filled  with  numerous  cells,  somewhat  strongly  pigmentcd,  but 


LESIONS  OP   PEEIPHEEAL  NERVES.  335 

normal  in  size.      The  pneumogastric   nucleus  is   rather   stroiio-ly 
vascular. 

Spinal  roots. — The  anterior  roots  of  the  cervical  région  are  nearly 
completely  destroyed.  It  is  with  difficulty  that  we  find,  at  long 
intervais,  a  tube  filled  with  medulla.  Elsewhere,  we  only  find 
empty  sheaths,  provided  at  very  regular  intervais  with  large  granular 
and  ovoid  nuclei,  and  pressed  ogainst  each  other  by  the  thickened 
endoneura.  This  state  is  exactly  the  same  as  that  of  the  anterior 
roots  of  the  dorsal  région.  In  the  lumbar  région^  we  scarcely  find 
any  atrophied  tubes.  The  posterior  roots  are  normal,  as  are  those 
of  the  hypoglossus. 

Feripàeral  nerves. — The  plirenic  nerve  of  the  left  side  and  two 
intercostal  nerves  hâve  only  been  examined.  Thèse  three  nerves 
hâve  undergone  analogous  altérations.  The  phrenic  nerve  the 
lésions  of  which  we  are  about  to  describe,  received  a  more  spécial 
examination.  One  portion  of  this  nerve,  taken  from  about  the  middle 
part  of  the  pericardium^  was  placed  in  osmic  acid,  then  one  part  was 
examined  by  dissociation  ;  another  was  hardened  by  alcoholic  gum 
which  allowed  sections  to  be  made.  Generally  speaking,  we  find  hère 
the  same  altérations  as  in  the  anterior  roots  ;  it  bas  merely  arrived 
at  a  less  complète  degree  of  development. 

The  longitudinal  sections  already  enable  us  to  detect  the  disap- 
pearance  of  a  large  number  of  tubes,  and  show  broad  connective 
bands,  strewn  with  numerous  nuclei,  separating  those  which  survive 
(PI.  YII,  fig.  5).     On  the  transversal  sections,  thèse  connective 
bands  appear  under  the  form  of  round  dises,  or  little  polyo-ons 
very  like  what  is  seen  on  a  section  of  bundles  of  fasciculated  con- 
nective tissue.     Carminé  tints  them  a  rose  colour,  and  in  one  point 
of  their  surface  a  black  spot  is  seen  from  time  to  time,  which  is  the 
section  of  a  meduUated  tube.     It  seems,  however,  that  the  atrophy 
does  not  invade  the  nerve  in  an  absolutely  irregular  manner  that  it 
proceeds  with  a  certain  degree  of  order  and  destroys  it,  so  to  speak 
by  fascicle.     In  fact,  the  tubes  preserved  are  grouped  beside  each 
other  and  form  islets,  which  contrast,  by  their  colour,  with  the  other 
points  of  the  préparation  where  the  destruction  took  place  (PI.  VII 
fig.  I,  a). 

Thèse  cross  sections  furnish  other  information  yet  :  they  allow  us, 
when  compared  with  similar  sections  of  a  healthy  nerve,  to  form  a 
good  idea  of  the  number  of  tubes  which  hâve  disappeared  (more  thau 
two  thirds  are  certaiuly  absent),  at  the  same  time,  we  see  the  consi- 


336  PROTOPATHIO  MUSOULAR  ATROPHY. 

derable  diminution  in  its  diameters  whicli  the  nerve  has  undergone— 
a  diminution  of  about  a  third.  The  préparations  by  dissociation, 
also,  enable  us  to  form  an  idea  of  the  process  which  has,  apparently, 
presided  over  the  atrophy  of  the  nerve.  We,  in  fact,  meet  with 
some,  but  very  rare,  tubes  analogous  to  that  of  which  we  bave  given 
a  sketch  (PI.  VII,  fig.  3). 

Thèse  tubes  hâve  preserved  their  normal  size,  but  the  axis-cylinder 
has  disappeared,  or,  at  least,  is  no  longer  visible,  the  medulla  is 
fragmented,  and  in  the  intervais  between  the  lumps  formed  by  it, 
we  note  the  existence  of  nuclei,  either  isolated  or  collected  together 
to  the  number  of  two  or  three,  evidently  contained  in  Schwann's 
sheath — the  signification  of  which  from  the  view-point  of  parenchy- 
matous  neuritis  could  not,  we  believe,  be  an  instant  dubious. 

Muscles. — A  certain  number  of  muscles  hâve  been  examined  by 
means  of  the  différent  methods  at  présent  in  use.  In  ail,  the  pré- 
dominant lésion  is  a  simple  atrophy  of  the  primary  fascicles,  with  a 
purely  relative  augmentation  of  the  interstitial  connective  tissue, 
and  the  absence  of  ail  exaggerated  production  of  adipose  tissue. 

The  unsound  fibre  usually  préserves  to  the  last,  its  cross  strise, 
and  generally  no  pigmentation  takes  place  in  the  muscular  nuclei, 
properly  socalled. 

However,  some  exceptions  to  this  gênerai  rule  are  met  with. 
Thus,  in  the  diaphragm,  and  in  the  spinalis  dorsi,  certain  fibres  are 
filled  with  granular  contents,  and  in  others  the  muscular  substance 
is  interrupted  at  intervais  by  clusters  of  nuclei  to  the  number  of  five,. 
ten,  and  sometimes  more.  Still  there  are  exceptional  cases.  An 
examination  of  longitudinal  sections  taken  at  différent  heights,  from 
thèse  muscles,  enable  us  to  form  a  more  précise  idea  of  the  extent 
of  thèse  lésions. 

The  latissimus  dorsi,  for  instance,  is  almost  entirely  converted 
into  a  thin  lamina  of  connective  tissue.  It  is  with  difficulty  that  a 
muscular  fibre  is  found  at  wide  intervais,  and,  singular  to  say,  this 
single  fibre  left  there  as  if  to  attest  the  existence  of  the  muscle,  is 
occasioually  voluminous.  The  trapezius,  in  its  lower  portion,  and 
the  reclus  abdominis  are  almost  as  profoundly  smitten  also.  As 
to  the  diaphragm,  which  has  most  especially  attracted  our  attention^ 
on  aocount  of  the  leeion  of  the  nerve  with  animâtes  it,  the  altération 
is  very  far  from  being  equally  advanced.  The  muscular  fibres  are 
still  numerous  hère  ;  they  hâve  preserved  their  striée,  and  at  first 
sight,  it  seems  difficult  to  admit  that  this  muscle  can  be  the  seat  of 


EVOLUTION    OF    LESIONS.  337 

grave  altération.  But  when  we  compare  with  sections  taken  from 
it  other  sections  taken  from  a  healthy  muscle  and  prepared  in  a  like 
manner,  the  différence  becomes  striking  by  tlie  fact  of  this  simple 
comparison  (PI.  VIII,  figs.  4  and  5). 

In  one,  the  fibres  are  large,  from  4.^/j.  to  90^  in  size,  ail  nearly  equal, 
and  barely  separated  from  each  other  by  a  little  connective  tissue. 
They  are  exactly  parallel.  In  the  other,  they  are,  generally  speaking, 
extremely  reduced  in  size,  of  from  ^fx  to  ^^fx,  of  very  unequal 
dimensions,  and  separated  from  each  other  by  comparatively  large 
connective  spaces.  The  latter  circumstance  explains  the  wavy  form 
which  they  hâve  assumed,  owing  to  the  fact  that  they  could  not 
foîlow  the  connective  fibres  which  retreated  under  the  influence  of 
.reagents. 

The  above  case  reproduces  exactly  enough,  as  will  be  seen, 
the  principal  clinical  characters  which  we  hâve  assigned  to  proto- 
pathic  spinal  muscular  atrophy  :  long  duration,  slow  évolution  ; 
absence  of  ail  spasmodic  phenomena,  attested  by  the  gênerai 
flaccidity  of  the  upper  extremities,  the  mobility  of  the  large  joints, 
the  patient^'s  ability  long  to  perform  partial  voluntary  movements 
enabling  her  to  secure  a  certain  object.  The  fingers  alone  were 
ilexed  and  their  articulations  rigid  ;  but  this  rigidity  only  super- 
vened  in  the  course  of  time  and  owing  to  prolonged  immobility. 
We  would  say  as  much  of  the  attitude  of  the  head,  which  was  only 
produced  slowly,  in  proportion  as  the  muscles  of  the  nape  of  the 
Deck  became  incapable  of  upholding  its  weight.  The  integrity  of 
the  lower  limbs  is  a  not  less  remarkable  fact,  since  the  patient  still 
■walked  easily  enough  even  the  day  before  her  death. 

However,  some  unwonted  phenomena  were  exhibited  ;  there  were 
pains  in  certain  parts  of  the  body,  theu  jerks  which  occurred  in  the 
lower  limbs.  The  latter  symptom  bas  evidently  but  little  import- 
ance^  for  it  took  place  long  before  the  invasion  of  the  affection  ; 
moieover,  it  only  happened  at  long  intervais,  so  rarely  that  no 
physician  ever  had  the  opportunity  of  observing  it.  It  has,  how- 
ever, been  noted  that  the  lésion  was  not  exactly  limited  to  the  sub- 
stance of  the  anterior  cornua;  it  had,  on  passing  through  the 
radicular  fascicles  produced  a  certain  degree  of  meningitis  and  a 
shght  cortical  sclerosis.  Hence,  it  is  natural  enough  that  thèse 
incidents,  in  the  anatomical  order,  should  be  translated  during  life 
by  some  exceptioual  symptoms  which  cannot  modify  in  any  essential 
particular  the  gênerai  aspect  of  the  cliuical  picture. 

VOL.  II.  22 


338  PROÏOPATHIC   MUSOULAR   ATROPHY. 

It  is  now  our  duty  to  show  that  a  study  of  the  lésions  leads  to 
similar  conclusions.  We  will  admit^  at  once,  witliout  staying  to 
reproduce  tlie  arguments  in  favour  of  tliis  opinion — arguments 
wliich  hâve  already  been  several  times  statgd  in  the  '  Archives  de 
Physiologie' — that  the  lésions  observed  in  the  muscles,  the  nerves, 
and  the  roots,  are  only  produced  consecutively  to  the  spinal  altéra- 
tion. As  to  the  sclerosis  of  the  anterior  radicular  zones,  the 
examination  of  a  certain  number  of  sections  of  the  spinal  cord 
suffices  to  show  that  this  is  an  accessory  lésion,  varying  in  extent 
from  one  section  to  the  next,  and  largely  dépendent  on  the  irritation 
propagated  by  the  anterior  radicular  filaments  during  their  intra- 
spinal  course.  It  is  most  marked  in  the  points  where  they  are  most 
numerous  ;  lastly,  it  takes  the  form  of  scierons  barids,  extended 
from  the  anterior  cornu  to  the  periphery  of  the  cord,  thus  very 
exactly  recalling  the  direction  of  the  nerves  which  are  being  de- 
stroyed. 

The  points  where  it  takes  a  more  considérable  extension,  where 
it  forms  around  the  anterior  cornu  a  zone  of  some  width,  are  pre- 
cisely  those  where  the  anterior  grey  myelitis  attains  its  maximum 
of  intensity  ;  ail  the  éléments  of  the  région  are  altered,  at  this 
level,  and  we  may  admit,  either  that  the  irritation  of  the  neuroglia, 
which  is  manifest  in  thèse  points,  lias  propagated  itself  gradually 
to  a  certain  distance  from  the  principal  focus  ;  or  else,  as  is 
more  probable,  that  this  irritativc  process  lias  been  transmitted  to 
the  radicular  zone  by  means  of  the  nerve-fibres  which  it  receives 
from  the  anterior  cornu. 

Pinally,  and  this  last  reason  is  a  most  important  one,  we  again 
find  nerve-cells  in  process  of  destruction,  in  the  lumbar  région, 
when  the  anterior  radicular  zones  are  found  perfectly  normal  in  that 
région. 

As  to  the  slight  degree  of  meningitis  which  is  observed,  ifc  is 
very  uatural  to  admit  that  it  is  jnoduced  under  the  influence  of  the 
lésion  of  the  radicular  filaments  on  their  passage  through  the  pia 
mater  ;  and  that  the  latter,  once  inflaraed,  bas  become  the  cause  of 
the  cortical  sclerosis.  If  now  we  consider  that,  in  certain  points — 
in  the  upper  part  of  the  cervical  région,  for  instance,  as  well  as  in  tliu 
dorsal  région, — the  nerve-cells  are  again  greatly  altered,  even  when 
the  interstitial  tissue  only  exhibits  minute  lésions,  we  shall  be  led  to 
the  belief  that,  in  ail  probability,  the  gangliouic  élément  was,  in 
this  case,  the  prima ry  seat  of  the  disease. 


LESIONS    OP    NEUVES   AND   MUSCLES.  339 

This  lésion  oftlienerve- élément  should  be  of  an  irritative  nature, 
at  least  if  we  are  to  judge  by  tlie  character  of  the  altérations  which 
it  provokes  when,  later  on,  through  its  influence  the  interstitial 
tissue  is  invaded  in  its  turn.  Tliickening  of  the  walls  of  the 
large  vessels,  multiplication  of  the  nuclei  of  the  capillaries,  pro- 
lifération of  the  cells  of  the  neuroglia,  sclerosis  of  the  white  columns 
in  the  spots  where  they  are  invaded — ail  thèse  phenomena  very 
evidently  dépend  upon  a  chronic  irritative  process. 

Por  ail  thèse  reasons,  therefore,  it  is  quite  legitimate  to  class  the 
cases  which  occupy  us  in  the  category  of  primary  anterior  chronic 
myélites,  and  more  particularly  of  chronic  parenchymatous  tepliro- 
myelitis. 

As  to  the  exaggerated  pigmentation  of  the  nerve-cells,  although 
that  may  not  be  a  necessary  fact,  since  it  lias  sometimes  been 
absent,  it  is  none  the  less  one  of  the  most  habituai  expressions  of 
the  organic  détérioration  of  thèse  éléments,  and  on  that  account 
deserves  spécial  mention. 

It  has  been  seen  that,  with  respect  to  the  peripheral  nerves,  those 
which  were  examined  showed  considérable  atrophy.  The  phenomena 
which  préside  over  this  atrophy  appear,  in  this  case,  to  be  idcntical 
with  those  which  are  produced  in  the  peripheral  extremity  of  a 
divided  nerve,  but  with  this  différence,  that  hère,  in  the  case  of 
amyotro])hy,  the  nerve  is  only  invaded  slowly  and,  as  it  were,  fibre 
by  fibre  (PI.  YIII,  fig.  i  and  fig.  3) . 

In  concluding,  it  is  right  to  remark  the  radical  altérations  under- 
gone  by  most  of  the  muscles  of  respiration,  particularly  the  inter- 
costals  and  the  diaphragm;  for  it  is  to  the  altération  of  thèse 
muscles  that  we  should  attribute  the  fatal  termination.  In  fact, 
there  was  no  apparent  lésion  of  the  lungs,  nor  of  the  heart,  and 
the  medulla  oblongata  can  scarcely  be  arraigned  hère.  Hence  it  is 
natural  to  admit,  in  order  to  explain  the  habituai  dyspnœa,  as  like- 
wise  the  symptoms  which  brought  the  life  of  the  patient  to  a  rapid 
close,  the  existence  of  progressive  paralysis  of  the  respiratory 
muscles.  The  number  of  fibres,  which  retained  their  functions, 
diminishing  day  by  day,  motor  impotence  would  hâve  developed  in 
a  parallel  manner,  till  the  day  came  when  the  lésion  had  grown 
gênerai  enough  no  longer  to  allow  of  the  regular  play  of  the  thoracic 
cage. 

Still,  to  explain  so  complète  a  paralysis  in  muscles  provided  with 
yet  numerous  fibres,  which  though  considerably  reduced  in  size  had 


510  PKOTOPATHIO    MUSCULAE   ATKOPHY. 

nearly  ail  retained  their  cross  striœ  (PI.  VIII,  fig.  5),  it  may  per- 
liaps  be  right  to  recognise  the  intervention  of  the  well-marked 
nerve-lesion,  particularly  so  far  as  the  phrenic  nerve  is  coucerned, 
which  for  the  most  part  left  them  in  the  condition  of  muscles 
paralysed  by  the  subtraction  of  nerve  action. 

Extract  from  the  '  Archives  de  Physiologie  normale  et  patholo- 
gique/ 1875,  p.  741. 


IV. 

TWO  CASES  OF  LATERAL  SYMMETEICAL  AMYOTROPHIC 
SCLEROSIS. 

(See  Lecture,  XII,  p.  i8o.) 

We  reproduce  hère  two  cases  which  completely  confirm  tlie  de- 
scriptions traced  by  M.  Charcot  in  liis  lectures  on  deiiteropathic 
amyotrophy.  They  were  published  by  M.  Charcot,  M.  Joffroy^ 
assisting  in  the  préparation  of  the  first,  and  M.  Gombault  in  that 
of  the  second." 

Case  I. 

Progressive  muscidar  atrophy,  especially  marked  i?i  the  upj^er  extre- 
miiies.  Atrophy  of  the  muscles  of  the  longue  and  orbiatlaris 
cris.  Paralysis  with  rigidity  of  the  lower  extremities.  Atrophy 
or  disappearance  of  the  nerve-cells  of  the  anterior  cornua  in  the 
cervical  and  dorsal  régions.  In  the  lulbus,  atrophy  and  de- 
struction of  the  nerve-cells  of  the  hypoglossus  nucleus,  atrophy 
of  the  anterior  spinal  roots,  of  the  roots  of  the  hypoglossus  and 
of  the  facial  nerve.  Symmetrical  rihand  sclerosis  of  the 
latéral  coliimns. 

Catherine  Aubel  was  admitted  to  the  Salpêtrière  (M.  Charcot^s 
wards),  in  the  month  of  June,  1865;  she  then  presented,  in  a 
marked  degree,  the  symptoms  of  progressive  muscular  atrophy,  the 
beginning  of  which  dated  back  for  about  nine  months  at  that  time» 

Her  relations,  brothers  and  sisters,  five  in  number,  had  had  no 
disease  worthy  of  notice,  and  ail  enjoyed  good  health.  Of  a  lym- 
phatic  tempérament,  she  had  glandular  enlargements  in  her  child- 
hood  ;  some  glands  even  suppurated,  and  her  neck  is  marked  by 
numerous  characteristic  scars,  Her  menses  hâve  been  regular  since 
the  âge  of  eleven. 

Her  state  of  health  does  not  ofFer  anything  noticeable  until  the 
âge  of  x^,  from  which  time  she  dates  the  beginning  of  the  présent 
affection.  On  the  2nd  September,  1864,  having  gone  her  full  time, 

'  '  Archives  de  Physiologie  normale  et  pathologique,'  1869,  p.  356. 
'  Ibid,  187 1 — 1872,  p.  509. 


3-1-2        LATERAL    SYMMETRICAL  AMYOTROPHIG   SCLEROSIS. 

slie  was  delivered  of  a  cliild,  wliich  lias  ever  siuce  enjoyed  good 
healtli.  Ou  tlie  6tli  September,  tlie  patient  says  that,  liaving  tried 
to  get  up,  she  found  it  impossible,  as  her  legs  were  too  weak  to 
support  \\ev,  and  appeared  paralysed.  On  tlie  1 2tli,  a  uew  attempt 
to  rise  from  bed  had  tlie  same  fate  ;  walking  and  standing  are 
almost  impossible,  owing  to  tlie  weakness  of  the  lower  extremities. 
About  the  sotli  September,  she  felt  pains  in  her  hands,  and  from 
tins  time  forth  her  upper  extremities  in  their  turn  became  progres- 
sively  weaker. 

Towards  the  ist  of  October,  she  was  "  taken  in  the  tongue/^ 
according  to  lier  own  expression,  and  her  utterance  began  to  grow 
very  mucli  embarrassed.  The  patient  then  went  on  foot,  as  well  as 
she  could  travel,  to  the  Hôpital  Saint-Antoine.  Sent  away  because 
there  was  no  vacancy,  she  returned  on  the  iith,  but  this  time  the 
lower  extremities  liad  become  too  weak  to  allow  her  to  walk  and  she 
wasobliged  to  go  in  a  car.  Atlmitted  on  the  same  day,  she  was  at 
once  put  under  a  course  of  nitrate  of  silver,  administered  in  pills. 
This  treatmoit  was  suspended  at  the  end  of  tliree  weeks.  It  had 
not  impeded  in  the  least  the  course  of  the  disease  ;  on  the  contrary, 
the  weakness  of  the  upper  and  lower  extremities  had  rapidly 
advanced;  walking  had  become  quite  impossible;  the  voice  had 
taken  a  peculiar  nasal  sound  {ïiasïllardê)}  her  utterance  was 
embarrassed,  difficult,  almost  unintelligible. 

It  was  impossible  for  us  to  ascertain  from  the  patient  the  epoch 
at  which  began  the  characteristic  atrophy  and  déformation  of  the 
upper  extremities  which  she  presented,  in  a  marked  degree  already, 
when  she  entered  the  asylum.  However  this  may  hâve  been,  at  the 
time  she  was  admitted  to  the  Salpêtrière,  the  affection  seemed  to 
hâve  entered  on  a  stationary  period,  and  no  aggravation  of  the 
symptoms  were  observed  from  the  month  of  June  to  the  iith 
September,  1865,  at  which  date  the  foUowing  note  was  taken: 
*'  The  face  is  still  covered  with  a  very  marked  mask.  The  phy- 
siognomy  bas  a  singular  expression  :  whilst  the  brow,  the  eyebrows, 
and  the  upper  part  of  the  cheeks  hâve  retained  their  mobility,  it  is 
remarked  that,  except  at  the  moments  when  the  patient  expériences 
a  somewhat  lively  émotion,  the  lower  part  of  the  face  remains,  as  it 

'  The  voice  is  said  to  bc  nasillarde  wlien,  the  mouth  beiug  open  and  the 
exterior  nares  closed,  it  appears  to  sound  withiu  and  t.hrougliout  tho  nasal 
cavities.  It  is  nasonnée  when,  mouth  and  nostrils  reinaining  IVee,  it  resounds 
iu  the  poslerior  portion  of  the  nasal  cavities  into  whicli  it  is  directed. — S. 


STMPTOMS.  343 

were^  motionless  and  lifeless.  But,  when  slie  lauglis  or  weeps^  tlie 
labial  commissures  are  very  strongly  drawn  back,  the  moutli  opens 
very  widely,  and  the  naso-labial  furrow  appears  exaggerated.  The 
patientj  however,  eau  shut  lier  mouth  firmly  enough,  and  peut,  but 
she  cannot  whistle,  blow,  or  simulate  the  act  of  kissing. 

She  seems  very  intelligent,  and  appears  to  understand  ail  the 
questions  put  to  her  ;  but  she  only  answers  with  the  greatest 
difficulty,  and  in  an  almost  unintelligible  manner.  The  voice  is 
nasal  [nasonnée)  :  speech  is  accompanied  by  a  sort  of  grunting,  and 
the  articulation  of  most  words  is  accomplished  slowly,  laboriously, 
with  extrême  trouble.  Speech  becomes  somewhat  less  indistinct 
when  the  patientas  nostrils  are  closed. 

The  tongue  is  small,  shrunken,  as  if  covered  with  convolutions 
on  the  dorsal  surface_,  which  is  the  seat  of  almost  incessant  fibril- 
lary  and  vermicular  movements.  It  cannot  be  raised  towards  the 
palate,  but  it  can  be  put  out  between  the  teeth,  but  with  difficulty. 
It  is  almost  impossible  for  the  patient  to  elongate  it  to  a  point,  or  to 
hollow  it  like  an  augur.  The  saliva  gathers  in  the  mouth,  and  is 
continually  flowing  out.  The  vélum  palati,  the  uvula,  présent 
their  normal  appearance,  and  when  a  spoon  is  pushed  back  into 
the  throat,  the  palate  rises,  but  indeed  rather  slowly. 

Eor  some  days  past,  Catherine  expériences  a  sensation  of  con- 
striction  in  the  pharyngeal  région,  though  direct  examiiiation  does 
not  discover  any  redness  in  the  mucous  membrane,  nor  any  swell- 
ing  of  the  amygdalse. 

Déglutition  is  sometimes  difficult,  and  it  happens  that  portions  of 
the  food  enter  the  larynx  and  cause  lits  of  suffocation  ;  but  neither 
drink  nor  food  ever  return  by  the  nostrils,  nor  does  solid  food 
accumulate  between  the  cheeks  and  the  dental  ajrch. 

The  movements  of  the  chest  seem  normal.  Auscultation  shows 
nothing  pathological  neither  in  the  heart,  nor  in  the  lungs,  and  ail 
the  functions  of  organic  life  are  accomplished  in  a  normal  manner. 

Siaie  of  limhs. — The  upper  extremities  are,  takeu  altogether, 
remarkably  emaciated  and  weakened,  they  hang  down  beside  the 
body  ;  but,  in  addition,  at  the  shoulder,  forearms,  and  hands,  there  is 
prédominant  atrophy  in  certain  muscles,  or  sets  of  muscles.  The 
deltoid,  on  either  side,  is  much  wasted,  and  the  shoulder  promi- 
nence  is  lacking.  In  the  forearms,  the  atrophy  affects  both  the 
flexor  and  extensor  muscles  of  the  fingers  ;  in  the  hands,  the  thenar 
and  hypothenar  eminences   are  remarkably   effaced  ;    the   palmar 


344       LATEEAL    SYMMETEICAL    AMYOTROPHIO  SCLEROSIS. 

hollow  is  excavated  owing  to  wasting  of  tlie  interossei;  moreover, 
the  fingers  are  ratlier  strongly  and  permanentlj  flexed,  especially  at 
the  joints  of  the  first  phalanges  ;  so  we  hâve  a  fine  example  of  the 
déformation  known  as  a  claw  or  talon  hand  {main  en  griffe) .  The 
movements  of  the  différent  parts  of  the  upper  extremities  are,  besides, 
extremely  restricted.  The  patient  can  scarcelj  raise  her  hands 
from  her  knees,  where  they  usually  rest  almost  inertly  ;  this  move- 
ment  of  élévation,  which  seems  to  require  much  effort,  cannot  long 
be  maintained,  and  is  accompanied  by  a  sort  of  trembling  of  the 
hands,  especially  in  a  latéral  direction,  which  is  very  singaiar. 
The  movements  of  flexion  and  extension  of  the  fingers  are  very 
restricted.  Since  the  month  of  January,  the  patient,  who  knows 
how  to  Write,  cannot  hold  a  pen  ;  her  hands,  besides,  are  of  no  use 
to  her,  and  it  is  wholly  impossible  for  her  to  lift  her  food  to  her 
lips.  The  movements  of  her  shoulder,  those  of  the  forearm,  and 
those  of  the  arm  are  also  much  restricted.  Generally  speaktng, 
the  left  upper  limb  is  perliaps  a  little  less  weak  than  the  right, 
There  is  no  sign  of  any  alteralion  of  sensibiliiy  whatever  through- 
out  the  whole  extent  of  the  upper  extremities, 

The  patient  can  neither  walk  nor  stand  alone,  Aasisted  by  two 
persons,  if  she  tries  to  take  a  few  steps,  her  legs  stiffen,  cross  each 
other,  and,  at  the  same  time,  her  feet  turn  in,  owing  to  an  involuu- 
tary  movement  of  forced  adduction. 

The  lower  limbs  are,  also,  greatly  emaciated  j  but  this  is  a  gênerai 
emaciation.  We  do  not  find,  as  in  the  upper  limbs,  déformations 
owing  to  prédominant  wasting  of  certain  sets  of  muscles.  The  feet 
are  a  little  rigid,  in  semi-extension,  and  strongly  turned  in. 

There  is  also  rigidity,  contracture,  in  the  knees,  which  are  half 
flexed,  and  in  adduction  ;  the  hips  likewise  appear  a  little  rigid: 
Muscular  strength,  however,  is  not  completely  abolished  in  the 
lower  extremities,  and  the  patient  can  fiex  a  little  and  extend  her 
legs.  Thèse  movements,  which  are  very  limited  in  extent,  are  not 
accompanied  by  tremulation. 

No  sensory  disorders  exist  in  the  lower  limbs,  where  the  patient 
feels  neither  pains,  nor  cramps,  nor  formication.  Electro-muscular 
sensibility  appears  normal,  whilst  it  seems  rather  exaggerated  in 
the  upper  extremities. 

Al  large  number  of  muscles,  especially  those  of  the  supedor 
extremities,  are  the  seat  of  extremely  marked  fibrillary  contractions. 
Thèse  are  particularly  noticeable  in  the  forearms  and  hauds.     They 


SYMPTOMS.  345 

come  on,  sometimes  spontaneously,  sometimes  under  the  influence 
of  a  touch.  They  are  strong  enougli  to  produce  very  marked 
extension  movements  of  the  fingers,  and  of  the  whole  hand.  When  a 
slight  tap  is  given  with  the  finger  to  the  dorsal  surface  of  the  fore- 
arm,  an  extension  movement  is  made,  which  is  soon  succeeded  by  a 
flexion  movement,  and  this  is  repeated  three  or  four  times,  after  a 
single  stimulation. 

If  the  forearm  be  placed  in  pronation,  by  striking  the  supinator 
muscle  a  slight  blow,  we  raay  détermine  a  movement  of  supination, 
owing  to  which  the  hand  is  turned  over  on  its  posterior  surface. 
Electric  exaltation  détermines  thèse  same  fibrillary  contractions  in  a 
still  more  marked  manner.  Thèse  spontaneous  or  provoked  fibril- 
lary contractions  are  also  remarked  in  ail  the  muscles  of  the  upper 
part  of  the  trunk.  They  are,  likewise,  very  marked  in  the  muscles 
of  the  neck,  but  they  are  most  fréquent  and  most  marked  in  the 
sterno-cleido-mastoid  muscles,  particular  in  that  of  the  left  side. 

No  trace  of  fibrillary  movements  is  seen  in  the  varions  muscles 
of  the  lower  extremities.  We  hâve  already  mentioned  that  they 
are  very  évident  in  the  tongue.  The  most  emaciated  muscles, 
those  of  the  forearm,  for  instance,  bave  preserved  electric  contrac- 
tility  in  a  high  degree.  Some  of  the  lower  extremities  also  contract 
under  the  influence  of  faradisation. 

Such  was  the  state  of  the  symptoms  in  the  month  of  September, 
1865,  one  year  after  the  beginning  of  the  disease.  Prom  that 
period  to  the  month  of  February,  1869,  no  noteworthy  change 
took  place.  The  numbness  of  the  upper  limbs  simply  increased, 
but  impotence  was  not  complète,  and  the  patient  could  still  move 
ber  Angers  a  little.  Contracture  of  the  lower  limbs  bas  also  made 
progress,  but  never  to  any  excessive  extent.  Finally,  atrophy  of 
the  muscles,  becoming  more  and  more  marked,  rendered  still  more 
characteristic  the  déformations  of  the  upper  limbs,  and  particularly 
those  of  the  hands. 

The  motor  impotence  and  atrophy  hâve,  likewise,  only  proceeded 
very  slowly  as  regards  the  face,  and  sub-hyoidean  région.  Never- 
theless  difficulty  of  pronunciation  and  ail  the  other  phenomena, 
recalling  the  picture  of  labio-glosso-pharyngeal  paralysis,  had 
become  worse,  whilst  no  noteworthy  disturbance  of  the  respiratory 
functions  were  superadded. 

In  the  trunk  no  new  symptoms  had  appeared.  The  emaciationi 
was  considérable,  but  without  any  évident  sign  of  muscular  atrophy. 


346       LATEEAL    SYMMETEICAL  AMYOTROPHIC    SCLEROSIS. 

Tlie  respiratory  muscles  acted  iiormally,  and,  in  particular,  there 
was  no  sign  of  diapliragmatic  paralysis. 

The  lower  extremities  exhibited  the  same  weakness,  the  same 
emaciation  already  described.  No  atrophie  déformation  of  dif- 
férent sets  of  muscles,  nor  fibrillary  contractions  were  observed. 
The  feet  still  retained  their  vicions  attitude,  they  were  turned  in 
and  slightly  extended  on  the  leg.  It  had  been  remarked  that  the 
patient  was  growing  weaker,  and  was  coughing  for  some  time, 
when  on  the  5th  of  JTebruary,  1869,  during  the  evening  visit  of  the 
house  physician,  she  was  found  in  a  rather  grave  state  of  asphyxia, 
which  had  almost  suddenly  shown  itself.  The  puise  was  at  136, 
there  were  50  inspirations  per  minute.  A  moist  laryngo-tracheal 
râle  appeared,  extending  to  a  great  distance.  The  upper  part  of 
the  respiratory  tubes  was  the  seat  of  a  considérable  accumulation 
of  mucous  matter,  which  the  patient  could  not  get  off.  Next  day 
thèse  symptoms  appeared  to  be  partially  dissipated  ;  but,  in  the 
evening,  they  returned  with  ail  their  gravity.  The  patient  suc- 
cumbed  in  the  evening  of  the  iith  Eebruary. 

Âutops^. — February  I3th,  1869.  a.  a.  Cadaveric  rigidity  was 
several  times  observed  in  this  case  ;  it  was  complète  twelve  hours 
after  her  death,  when  the  first  examination  of  the  body  was  made. 
It  persisted  thus  throughout  the  entire  day,  on  the  1 2th,  and  still 
very  manifestly  existed  on  the  morning  of  the  I3th.  It  was  very 
strong  even  in  the  upper  limbs,  where  atrophy  was  most  marked. 
Before  making  an  autopsy,  the  circumference  of  her  wrists,  arms, 
and  legs  was  measured,  and  the  foUowing  figures  were  the  resuit  : 

Circumference  of  wrist o"i25  m. 

„  arm      .         .         .         .         .         .  o'i7 

„  mid-thigli      .....  o'365 

„  leg,  a  little  above  the  malleoli         .  0-175 

There  was  no  différence  between  the  members  on  the  right  side 
and  those  of  the  left. 

b.  Thoracic  cavity. — The  lungs  présent  tubercular  granulations, 
in  the  inferior  lobe  of  each,  and  nuclei  of  incipient  caseous  pneu- 
monia.  The  summits  were  healthy.  The  heart  weighed  185 
grammes.  Its  tissue  was  red,  firm,  and  it  appeared  entirely  sound. 
There  was  no  valvular  lésion. 

The  other  viscera  offered  nothing  noticeable. 

c.  Miiscular    System. — Dissection    of    the    muscles    gave    the 


ATJTOPST.  347 

following  results  :  i°.  Face.— The  muscles  of  ilie  clieeks  and 
chin,  but  cliiefly  the  buccinato- labial  muscles  were  atrophied,  pale, 
yellow,  reduced  to  tliin  muscular  slips.  The  orbicularis  palpebra- 
rum,  tlie  frontal,  temporal,  and  masseter  muscles  preseuted  notliing 
abnormal. 

3°.  Nccl-. — The  sterno-cleido-mastoid  muscles  appeared  healthy. 
The  muscles  of  tlie  supra-hyoidean  région  are  very  small.  They 
présent  a  yellow  dead-leaf  colour  at  the  point  of  the  tongue.  They 
are,  on  the  contrary,  ratlier  red,  but  manifestly  wasted,  at  the  base 
of  this  organ. 

3°.  Ujjper  extremities. — The  deltoid  is  atrophied  in  a  very  marked 
manner;  it  is  thin,  of  a  pale  yellow  dead-leaf  hue.  The  muscles  of 
the  arm  are  small,  but  of  an  almost  normal  red  colour.  In  the 
forearm,  the  muscles  are  exceedingly  slender,  but  the  red  colour  is 
sufficiently  well  preserved.  The  muscles  of  the  hand  are  a  dead- 
leaf  yellow,  and  much  wasted,  especially  the  interossei.  The 
muscles  of  the  hand  are  certainly,  with  tliose  of  the  tongue,  the 
most  altered. 

4°.  Tninh. — The  sacro-lumbar  muscular  mass,  in  its  lower  part, 
seems  to  hâve  undergone  a  certain  degree  of  atrophy.  Its  colour 
is  yellowish.  The  muscles  of  the  abdomen  présent  the  same 
characters  ;  and  with  respect  to  them,  as  well  as  those  of  the  back, 
the  lésions  seem  to  diminish,  and  even  to  disappear,  as  we  a^proacli 
the  breast.  The  pectorals  are  red,  and  do  not  exhibit  much 
marked  atrophy.  The  interossei  are  rather  thin,  and  somewhat  yel- 
lowish.   The  diaphragm  appears  healthy,  at  least,  to  the  naked  eye. 

5°.  Loioer  extremities. — The  emaciation  is  rather  marked  ;  the 
muscles  are  not  large,  but  yet  their  emaciation  is  not  excessive,  if 
we  consider  the  gênerai  emaciation  of  the  subject.  On  the  wliole, 
tliere  seems  hère  to  be  no  atrophy  properly  so  called.  The  muscles 
are  red,  and  their  tissue  appears  healthy. 

d.  Peripheml  nerve  System. — Viewed  with  the  naked  eye,  one  is 
struck  by  the  considérable  changes  which  are  produced  in  the  size 
and  colour  of  the  anterior  roots.  They  are  formed  by  the  union  of 
nerve-bundles,  reduced  almost  to  filaments,  so  that  they  are  ex- 
ceedingly slender.  Their  colour  lias  assumed  a  very  marked 
greyisli  hue,  without,  however,  exhibiting  that  serai-transparence 
whicli  is  seen  '\\\  nerves  which  hâve  undergone  complète  atrophy. 
Thèse  changes  of  size  and  colour  are  ail  the  more  striking,  because 
nothing  of  the  kiud  is  found  in  the  posterior  roots,  which  hâve  pre- 


348        LATERAL    SYMMETRICAL  AMYOTROPHIC  SCLE ROSIS. 

served  their  normal  volume  and  tlieir  white  colour.  lu  the  cervical 
région^  especially,  thèse  lésions  are  most  marked,  however  they  are 
seen  tliroughout  tlie  whole  heiglit  of  tlie  dorsal  région  ;  but  they 
tend  to  become  effaced,  as  we  go  away  from  the  cervical  région. 
In  the  lumbar  région^  the  anterior  roots  hâve  resumed  their  normal 
size  and  colour. 

The  facialis  and  hypoglossus,  also,  présent  a  greyish  hue,  analo- 
gous  to  that  of  the  anterior  cervical  and  dorsal  roots.  This  change 
in  colour  becomes  particularly  manifesta  when  we  compare  thèse 
nerves  to  the  others,  such  as  the  lingual,  for  instance,  which  hâve 
preserved  their  whitish  lustre.  No  diminution  in  the  size  of  thèse 
nerves  is  remarked,  analogous  to  that  exhibited  by  the  anterior  roots. 
The  other  peripheral  nerves  do  not  show  any  perceptible  modification. 

e.  Central  nerve  System. — The  encephalon  shows  no  altération. 
We  hâve  mentioned,  in  speaking  of  the  peripheral  System,  those  of 
the  cranial  nerves  which  presented  any  change  in  colour.  The 
cord,  examined  in  the  fresh  state,  lias  not  revealed  any  altération, 
perceptible  to  the  naked  eye,  in  the  greater  part  of  its  ex- 
tent,  but  for  about  5  centimètres  above  the  dorso-lumbar 
enlargement,  it  exhibited  excessive  diffluence.  Large  vessels, 
gorged  with  blood,  and  a  diffuse  red  tint,  were  observed  in  the 
softened  portion.  The  ramollissement  principally  affected  the  left 
side  aaid  the  posterior  part  of  the  cord.  It  is  possible  that,  in 
spite  of  ail  the  care  taken  in  removing  the  cord,  this  ramollissement 
may  hâve  been  produced  artificially  ;  we  shall  see  that  a  micro- 
scopic  examination  seems  to  lend  support  to  this  view. 

B.  Microscqpic  examination  :  Mmcidar  System. — We  shall  begin 
the  account  of  the  microscopic  examination  of  the  muscular  System 
we  made  by  the  description  of  the  muscles  of  the  hand  ;  thèse, 
indeed,  are  the  muscles  which  exhibit  the  most  advanced  lésions. 
The  muscles  of  the  thenar  and  hypothenar  eminences,  and  the 
interossei  had  reached  the  same  degree  of  degeneration,  and  gave 
the  same  results,  on  microscopic  examination.  The  préparations 
were  made  in  the  fresh  state.  We  shall  take  as  type  of  our 
description  the  changes  observed  in  the  right  opponens  pollicis. 

On  shredding  the  muscular  substance  with  needles  on  the  glass 
slip,  it  is  found  that  the  consistence  of  the  fibres  is  somewhat 
greater  than  usual  ;  it  recalls  that  of  eonnective  tissue.  In  most 
of  the  muscular  bundles  there  exist  fine  dark  granulations, 
becoming  brilliant  and  pearly  at  a  certain  focus,  which  seem  to  be 


MICROSCOPÎC    EXAMINATION.  349 

fatty  granulations;  neither  acetic  acid  nor  potash  dissolves  them. 
We  liave  repeated  thèse  tests  several  times,  and  the  resuit  has  been 
always  the  same.  Thèse  granulations  vary  much  in  number  and 
size,  in  différent  fibres.  The  transverse  and  longitudinal  strise, 
which  are  very  distinctîy  marked  in  certain  atrophied  but  only 
slightly  granulated  fibres,  are  more  or  less  completely  masked  in 
those  where  the  granulations  exist  in  abundance. 

The  striaî  hâve  entirely  disappeared  in  a  certain  number  of 
muscular  fibres,  which  resemble  cylinders  filled  with  transparent 
matter,  and  which  enclose  a  more  or  less  considérable  number  of 
granulations  that  are  generally  large  in  proportion  to  their  few- 
ness.  Thèse  granulations  are  no  more  dissolved  by  acetic  acid 
nor  potash  than  were  those  of  the  fibres  which  hâve  retained  their 
strire. 

The  size  of  a  number  of  the  muscular  fibres  seems  normal,  but 
mostly  we  remark  an  often  considérable  diminution.  Thus,  beside  a 
muscular  fibre  of  normal  size,  we  see  others  the  cross  diameter  of 
which  is  reduced  to  a  third  or  a  half.  Certain  fibres  even  présent  a 
diameter  four  and  sometimes  five  tiraes  smaller  than  the  normal. 
And  it  is  a  noticeable  and  important  peculiarity  to  see  a  large 
number  of  fibres,  which  hâve  undergone  au  atrophy  so  complète, 
still  exhibiting  very  distinct  striation,  and  beiug  scarcely  or  even 
not  at  ail  granular. 

Among  the  most  altered  muscular  fibres,  only  a  small  number 
hâve  shown  fragmentary  division  of  the  muscular  substance.  In  the 
fibres  where  we  observed  this  division,  the  lumps  of  muscular  sub- 
stance were  pressed  against  eacli  other  ;  very  rarely  they  hâve  an 
interval  between,  and,  then,  in  thèse  points,  the  sarcolemma  had 
shrunk  back.  We  hâve  not  found  any  multiplication  of  nuclei  in 
the  tubes  of  the  sarcolemma,  as  recently  remarked  by  M.  Hayem 
in  a  case  of  progresssive  atrophy  lately  published. 

The  vessels  in  the  afPected  muscles  did  not  reveal  any  altération. 
We  hâve  several  times  succeeded  in  very  distinctîy  seeing  the  liitle 
nerves  of  the  muscles  ;  we  did  not,  in  thèse  cases,  remark  that  they 
contained  any  degenerated  nerve-fibres.  The  interfihnllary  cov,' 
nective  iissue  appears  more  abundant  than  in  the  normal  state,  and 
an  exaggerated  proportion  of  rounded  or  fusiform  nuclei  are  per- 
ceived. 

In  addition  to  the  preceding  altérations,  most  of  the  muscular 
bundles  show  a  finely  shredded  appearance,  which  is  very  remark- 


350     LATERAL    SYMMETRICAL    AxVIYOTEOPHIC    SCLEROSIS. 

able  at  tlie  broken  ends  of  tlie  fibres  ;  tins  shredded  appearance 
is,  however^  also  met  witb  in  altérations  of  the  muscle  wliich  hâve 
nothing  in  common  with  progressive  atropliy  ;  thèse  are  commonly 
observed  in  the  muscles  of  the  lower  extremities  in  the  case  of 
individuals  who  hâve  long  remained  motionless. 

To  sura  up  :  in  the  muscles  of  the  hand,  that  is,  where  the 
lésions  vi^ere  most  marked,  we  hâve  observed  what  follows  :  i  '',  a 
diminution  of  volume  in  the  muscular  mass  ;  2°,  a  pale  yellow  hue 
of  the  muscles;  3°,  greater  consistency  of  the  muscle,  recalling 
that  of  connective  tissue  ;  4°,  granular  fatty  altération,  little  marked 
in  certain  fibres,  very  évident  in  others  ;  5°,  fragmentary  division  of 
the  muscular  substance  ;  6°,  atrophy  of  certain  muscular  fibres, 
simple  and  independent  of  ail  fatty  or  waxy  degeneration  ;  7°,  pro- 
lifération of  interfibrillary  connective  tissue. 

We  shall  conclude  this  abstract  by  pointing  out  that,  in  one  and 
the  sarae  préparation,  ail  thèse  altérations  might  be  seen  simul- 
taneously. 

Side  by  side,  with  an  entirely  healthy  or  but  slightly  granular 
muscular  fibre,  a  fibre  might  be  reraarked  whose  strise  were  ahnost 
completely  masked  by  fatty  granulations.  Beside  thèse,  others 
had  wholly  undergone  vitreous  degeneration  ;  others,  again,  showed 
every  degree  of  atrophy.  Some  presented  division  in  lumps  of  the 
muscular  substance.  In  the  intervais  between  thèse  fibres  vras 
seen  a  large  quantity  of  connective  tissue  and  rounded  or  fnsiform 
nuclei. 

With  respect  to  the  muscles  of  the  longue,  we  may  confine  our- 
selves  to  repeating  the  foregoing  description.  Let  us  only  observe 
that  the  lésions  were  most  marked  in  the  intrinsic  muscles  of  this 
organ.  The  muscles  of  the  forearm  hâve  nearly  preserved  their 
normal  colour.  However,  we  find  in  the  intervais  of  the  fibres  a 
marked  increase  in  the  connective  tissue  ;  there  also  are  granular 
fatty  fibres,  vitreous  fibres,  and  others  considerably  wasted,  but, 
generally  spcaking,  ail  thèse  lésions  are  mucli  less  marked  than  in 
the  hand.  In  the  deltoid,  we  discover  ail  the  most  advanced 
altérations  which  we  hâve  described. 

The  sterno-cleldo-mastoid  muscles  hâve  been  specially  examined. 
It  will  be  recollected  that,  in  the  observation,  they  were  mentioned 
as  being,  especially  the  left,  subject  to  fibrillary  contractions,  which 
are  remarkable  for  their  spontaneity,  frequency,  and  intensity.  The 
préparations  of  muscular  substance,  taken  from  the  left  sterno- 


LESIONS    OF   MUSCLES    AND   NEEVES.  351 

cleido-mastoid,  to  our  great  astonishment,  presented  absolutely  no 
altération  whatever.  The  fibres  were  remarkable  for  their  com- 
paratively  considérable  size,  their  distinct  striation^  and  the  absence 
of  ail  degeneration.  We  did  not  even  remark  in  thèse  muscles 
that  shredded  appearance  which  was  well-nigh  gênerai  in  the 
muscles  of  the  upper  and  lower  extremities. 

^\\& pectorales  did  not  exhibit  any  altération. 

The  intercostales  showed  only  a  slightly  marked  granular  fatty 
degeneration,  and  the  shredded  appearance.  The  same  is  to  be 
said  of  the  diaphragm,  where  we  only  met  with  a  small  number  of 
fibres  in  which  the  granulations  were  abundant  enough  to  mask 
the  cross  striœ. 

The  muscular  éléments  in  the  loioer  extremities  enclosed  few  or 
no  fatty  granulations.  They  are  not  perceptibly  atrophied;  the 
strise  are  clearly  marked,  and  they  ofFer  no  altération  other  than 
the  shredding. 

3°.  a.  Anterior  spinal  roots.  Examination  in  the  fresh  state. — 
The  number  of  the  nerve-tubes  which  hâve  preserved  their  normal 
character,  in  thèse  roots,  is  greater  than  might  be  supposed,  to 
judge  by  the  diminution  of  their  size,  and  the  greyish  hue  which 
they  exhibit.  However,  in  at  least  one  half  of  the  tubes,  we  can 
note  every  degree  of  atrophy,  from  simple  emaciation  to  com- 
plète disappearance  of  the  meduUary  cylinder.  In  thèse  tubes,  we 
nowhere  met  with  trails  of  fatty  granulations.  What  we  hâve 
said  relates  especially  to  the  cervical  région  of  the  cord  ;  in  the 
dorsal  région,  the  atrophie  lésions  are  less  marked,  especially  in  the 
lower  parts  of  this  région;  and,  on  a  level  with  the  lumbar  enlarge- 
ment,  they  are  completely  absent. 

h.  The  posterior  sonnai  roots  hâve  been  examined  in  comparison 
with  the  anterior  ;  no  trace  of  the  nerve-tubes  was  observed. 

c.  Cranial  nerves. — The  facial  and  hypoglossus,  examined  in  the 
fresh  state,  in  différent  parts  of  their  course,  presented,  especially 
the  last,  lésions  comparable  to  those  which  hâve  been  mentioned  iu 
référence  to  the  anterior  spinal  roots.  But  the  number  of  healthy 
tubes  was  relatively  greater.  The  lingual  and  the  pneumogastric 
nerves  hâve  been  the  subject  of  a  spécial  examination  ;  they  did 
not  appear  to  ofiîer  any  altération. 

d.  Rachidian  nerves. — The  two  phrenic  nerves,  especially  that 
of  the  right,  hâve  seemed  to  us  to  enclose  a  certain  number  of 
nerve-tubes  atrophied  in  différent  degrees.     Analogous  altérations 


352      LATEEAL    SYMMETRICAL    AMYOTKOPHIO    SOLEROSIS. 

were  remarked  in  tlie  médian  and  ulnar  nerves,  examined  in  the 
forearm  ;  in  the  latter  nerves,  some  atrophied  nerve-tubes  presented 
évident  granular  degeneration.  The  examination  of  the  (jreat  sym- 
jmtlieiic,  in  the  neck,  and  inferior  ganglia,  did  not  yield  any  décisive 
resuit. 

e.  Spinal  cord.  Examinatioii  of  sojïened  portion  ht  the  fresh 
state. — It  will  be  recollected  that,  immediately  above  the  lumbar 
enlargement,  the  cord  showed  a  remarkable  diffluence  over  a  certain 
extent  ;  fragments  of  nerve-tissue,  coming  from  this  softeued  spot, 
were  placed  under  the  microscope  immediately  after  the  autopsy  ; 
the  nerve-tubes  exbibited  the  characters  of  the  normal  state  ;  in 
the  intervais  which  they  left  between  them  we  saw  neither  granular 
bodies,  nor  fatty  granulations,  neither  did  the  sheaths  of  the  vessels 
enclose  any  granular  éléments. 

This  négative  resuit  ought  to  make  us  think  either  that  the  ra- 
mollissement was  of  quite  récent  date,  or  that  it  was  artificially 
produced. 

Examination  of  jireparations  hardened  hy  chromïc  acid  and 
colonred  hy  carminé.  Cervical  région. — The  examination  of  trans- 
verse sections,  taken  at  différent  heights,  shows  altérations,  some  of 
which  affect  the  antero-lateral  fascicles  of  the  cord,  and  others  the 
grey  substance,  particularly  the  anterior  cornua — they  are  nearly 
the  same  throughout  the  whole  extent  of  the  région. 

On  every  point  of  the  antero-lateral  columns,  the  septa  of  con- 
nective  tissue  hâve  assumed  considérable  importance;  they  are 
remarkably  thickened,  and  they  appear  to  hâve  multiplied.  In  the 
spaces  circumscribed  by  them,  as  they  anastomose  and  cross,  we 
readily  recognise  the  section-surfaces  of  nerve-tubes  which,  on  a 
level  witli  the  anterior  fascicles  and  in  the  anterior  portion  of  the 
latéral  fascicles,  hâve  almost  entirely  preserved  their  normal  dia- 
meter.  But,  in  a  part,  which  corresponds  to  •  the  most  posterior 
part  of  the  latter  fascicles  and  in  the  whole  extent  of  a  région 
which,  internally,  bounds  the  posterior  cornua,  whilst  externally  it 
extends  almost  to  the  cortical  layer,  the  connective  matrix  has  be- 
come  quite  prédominant.  The  nerve-tubes,  which  hâve  retained 
their  normal  diameter,  hâve  hère  become  very  rare;  most  of  the 
tubes  are  atrophied  in  différent  degrees,  and  a  large  number  of 
them  are  only  represented  by  an  axis-cylinder.  "When  the  sections 
are  examined  under  a  low  power,  the  points  where  the  sclerous 
altération  of  the  latéral  columns  thus  predominate  appear  under  the 


LESIONS  OP  SPINAL  CORD.  35S 

form  of  two  little  red^  transparent, irregularly  rounded  patclieSj  whicli 
are  placed  symmetrically  towards  the  raost  posterior  parts  of  tliese 
colurans,  immediately  external  to  tlie  posterior  grey  cornua.  The 
posterior  wliite  columns  exliibit  no  altération. 

In  the  examination  of  the  greymltdance,  tlie  liigli  degree  of  atrophj 
wliich  most  of  the  nerve-cells  hâve  undergone  in  the  anterior  cornua 
first  strikes  the  eye  ;  it  is,  also,  évident  that  a  certain  nuniber  of  thèse 
cells  hâve  disappeared  without  leaving  any  trace.  The  cells  of  the 
inner  or  anterior  group  are  those,  especially,  which  hâve  undergone 
the  deepest  altérations  ;  hère,  ail  the  cells  that  remain  are  more  or 
Icss  wasted^  whilst  in  the  outer  group,  in  most  of  the  préparations, 
we  see  one,  two,  three,  and  even  sometimes  four  of  them,  which  hâve 
nearly  completely  preserved  their  dimensions  and  ail  the  other 
characters  of  the  healthy  state.  Among  the  wasted  cells  some,  though 
six  or  seven  times  smaller  than  in  the  normal  state^  hâve  still  re- 
tained  their  stellate  form,  their  prolongations,  and  still  possess  a 
distinct  nucleus  and  nucleolus.  Others  are  only  represented  by 
small  irregiilar  angular  masses,  without  prolongations,  yellow, 
brilliant,  vitreous  looking.  In  such  cases  the  nucleus  is  generally 
no  longer  distinct.  Ail  thèse  altérations  may  be  clearly  discriminated 
when  the  injured  parts  are  compared  with  corresponding  parts  in 
sections  of  the  healthy  cord.  As  a  standard  of  comparison,  we  hâve 
taken  the  beautiful  préparations  which  we  owe  to  the  kindness  of 
Dr.  Lockhart  Clarke. 

The  connective  matrix  of  the  anterior  cornua  showed  itself  under 
the  appearance  of  a  finely  granulated  mass  ;  we  did  not  remark  that 
the  nuclei  of  the  neuroglia  were  more  abundant  than  in  the  normal 
state.  Matters  were  différent  in  the  anterior  and  posterior  com- 
missures ',  hère  the  nuclei  appeared  numerous,  especially  in  the 
neighbourhood  of  the  central  canal.  The  latter  was  completely 
obliterated  by  a  mass  of  epithelial  cells. 

In  the  substance  of  the  commissure,  as  in  the  anterior  cornua,  the 
vessels  exhibited  walls  manifestly  thickened,  at  times  covered  with 
numerous  nuclei.  The  posterior  cormui  of  grey  substance  appeared 
to  possess  ail  the  conditions  of  the  healthy  state. 

Dorsal  région. — Only  the  upper  two  thirds  of  this  région  could. 
be  examined.  The  sclerosis  of  the  latéral  columns  was  seen 
throughout  its  height,  at  least  as  well  marked  as  in  the  cervical 
région;  as  in  the  latter,  though  to  a  lesser  degree,  the  cells  of  the 
anterior  cornua  were  atrophied,  and  reduccd  to  a  hw. 

99. 


354.       LATERAL   SYMMETBTCAL    AMYOTUOPHIC   SCLEEOSIS. 

Lumhar  région. — The  symraetrical  sclerous  altération  of  tlie 
latéral  columns  is  also  very  rlistinctly  rnarked  hère,  but  yet  less 
extensively  than  in  tlie  other  régions  of  the  cord  ;  it  occupies  the 
same  position.  The  cells  of  the  anterior  cornua  are  alraost  normal 
innumber;  they  generally  présent  the  dimensions  of  the  healthy 
state.     Only  a  few  exhibit  wel!-marked  atrophie  lésions. 

Bulbar  région  :  Sections  made  above  the  calanms. — By  raeans  of 
cross-sections,  taken  at  différent  heights,  in  the  olivary  région  and 
beneath,  we  bave  been  able  most  distinctly  to  observe  that  the  cells 
of  the  origin-nucleus  of  the  hypoglossns  arc  geuerally  throughont 
the  entire  extent  of  thèse  nuclci,  profoundly  altered,  atrophied,  or 
even  destroycd.  This  altération  recalls  exactlj  that  which  was 
noted  in  référence  to  the  cells  of  the  anterior  cornua  of  the  cord,  in 
the  cervical  and  dorsal  régions.  We  hâve  talcen,  as  a  standard  for 
comparison  in  this  department  of  our  investigation,  some  very  fine 
sections  of  healthy  bulbi,  prepared  by  Dr.  Lockhart  Clarke.  We 
hâve  also  utilized  the  yet  unpublished  plates  of  the  '  Iconographie 
photographique'  of  M.  Duchenne  (de  Boulogne),  relative  to  the 
structure  of  the  bulbus.  Now,  on  Clarke^s  sections,  made  at 
about  half  a  centimètre  above  the  point  of  the  calamus  scriptorius 
and  representing  the  normal  state,  one  could  count  from  40  to  50 
tripolar  or  quadripolar  large  cells,  in  the  nucleus  of  the  hypoglossus, 
whicli  in  this  région  is  voluminous  and  well  defined  everywhere; 
on  the  other  hand,  on  sections  taken  from  our  patient,  we  could 
only  find  3  or  4  at  most  of  thèse  cells,  which  were  nearly  normal  ; 
shorn  the  others  had,  for  the  most  part,  totally  disappearcd, 

Some,  which  were  considerably  wasted,  could  still  be  discerned 
nnder  a  higli  magnifying  power  ;  others  were  only  represented  by 
small  irregular  masses,  of  an  ochreous  yellow  colour,  brilliant,  and 
of  their  prolongations. 

In  addition,  it  could  be  seen  that  the  délicate  tracts  (cell  pro- 
longations, probably),  which,  in  the  normal  state,  cross  and  recross 
in  a  thousand  directions  in  the  intervais  between  the  cells,  were 
completely  effaced  hère;  and  between  the  cells  nothiug  now  was 
found  save  an  amorphous,  fmely  granular  mass  ;  finally,  the  nucleus 
of  the  hypoglossus,  considered  as  a  whole,  appeared  to  hâve  lost 
its  rounded  outlines;  it  presented  an  oval  form  transverseîy,  and 
had  evidently  diminished  in  ail  directions.    , 

On  the  saûie  sections,  immediately  external  to  the  nucleus  of  the 
hypoglossus,  could  be  recognised  the  little  group  of  cells  which 


SECOND  CASE.  355 

Clark  connects  with  the  inferior  origins  of  the  facial.  Ail  tlie  cells 
were  healtliy,  and  seemed  normal  in  number. 

Stiil  more  external  is  found  the  orujin-nuclcus  of  the  p^ieumo- 
gastric.  Most  of  the  cells  of  the  group  were  untouched,  a  few  of 
them  onlj  (7  or  8  in  each  nucleus,  and  each  préparation),  and 
thèse  the  most  anterior,  exhibited  yellow  degeneration  to  a  very 
marked  extent,  or  else  had  undergone  a  very  remarkable  black 
pigmentation. 

Sections  iaken  at  the  point  of  the  calaimcs. — In  front  of  each  side 
of  the  central  canal  the  nuclei  of  the  hypoglossus  are  found.  There, 
also,  the  cells  are  wasted  or  degenerated.  Eehind  the  canal  and 
on  cither  side,  the  nuclei  of  the  spinal  nerve  may  be  studied  ;  both 
of  them  show  some  cells  which  hâve  undergone  yellow  degeneration 
or  black  pigmentation,  and  they  are  at  the  same  time  deformed. 
The  other  cells  of  thèse  nuclei  are  normal. 

Section  made  above  the  olivary  hodies. — The  origin-nuclei  of  the 
facial,  external  oculo-motor_,  and  auditory  nerves,  appeared  to  us  to 
présent  ail  the  characters  of  the  normal  state. 

Case  II. 

Bymwctrical  sclerosis  of  the  latéral  coliinms  of  the  cord  and  anterior 
p//ramids  in  hulhus.  Atrophy  of  the  cells  of  aoiterior  cormca 
qf  the  cord.  Progressive  musc lUar  atrophy.  Glosso-larynyeal 
paralysis. 

Eîizabeth  P — -,  aged  58^  entered,  July  11,  1871,  the  infirmary 
of  the  Saltpctrière  (wards  of  M.  Charcot). 

Data  supplied  tjy  her  son. — The  affection  with  which  she  is 
stricken  does  not  seem  to  hâve  begun  suddenly.  In  the  month  of 
July  last,  P —  wuxs  still  able  to  walk,  though  with  some  difficultv. 
lier  left  hand  was  already  useless,  and  was  kept  close  to  the  body. 
She  also  complaincd  of  finding  her  right  hand  becoming  weak  for 
some  time,  which  was  a  hindrance  to  her  taking  food.  She  had, 
likewise,  a  slight  difficulty  in  speaking,  but  déglutition  was  per- 
formed  with  ease. 

Présent  state:  2qth  Septemher,  1S71. — The  physiognomy  is 
stupid  ;  from  the  mouth^  which  is  always  wide  open,  the  saliva  is 
constantly  flowing. 

It  seems  as  if  ail  the  muscle  of  the  face  were  in  a  state  of  per- 


356      LATERAL   SYMMETRIOAL    AMYOTROPHIC   SOLEROSIS. 

manent  contracture^  whicli  becomes  exaggerated  whenever  th& 
patient  laug'hs  or  weeps  ;  tlie  sort  of  grimace  whicli  is  then  pro- 
duced  ouly  passes  away  with  extrême  slowness. 

The  movements  of  the  orbicularis  oris  are  notably  impeded. 
The  lips  cannot  meet  as  in  the  act  of  whistling  or  blowing.  In 
blowing  out  a  candie,  the  mouth  is  half  open  ;  she  succeeds  in  ex- 
tinguishing  it  even  when  it  is  placed  at  some  distance  from  her 
mouth.  Dicluction-movement  of  the  maxillœ  appears  impossible. 
Contraction  of  the  masticatory  muscles  is  of  but  little  strength, 
hence  she  can  only  triturate  food  when  it  is  soft. 

Articulation  of  words  is  abolished  ;  the  efforts  of  the  patient  only 
resuit  in  the  production  of  a  sort  of  grunting,  which  is  quite 
incompréhensible.  Intelligence  seems  preserved  to  some  extent, 
and  the  patient  seems  to  understands  ail  the  questions  put  to  her. 

The  tongue  is  stricken  with  almost  absolute  motor  impotence^ 
whilst  it  also  présents  ail  the  characteristics  of  an  already  far 
advanced  atrophy.  Small^  shrunken^  stirred  by  fibrillary  move- 
ments, ploughed  with  furrows,  and  habitually  covered  with  a 
blackish  fur,  it  remains  glued  to  the  floor  of  the  mouth,  and  it  is 
with  difficulty  carried  forward  and  a  few  millimètres  beyond  the 
lips.  As  to  the  movement  of  elevating  the  tip  towards  the  palatine 
arch,  this  is  totally  abolished. 

Difficulty  of  déglutition,  though  a  little  less  incomplète,  is  yet 
very  noticeable.  It  is  only  in  the  last  few  days  that  it  became 
suddenly  marked.  When  a  liquid  is  introduced  into  the  mouth, 
the  greater  part  of  it  flows  out  between  the  lips  ;  then  a  séries  of 
deglutition-movements  follows,  with  considérable  ascent  of  the 
larynx  and  very  sonorous  pharyngeal  noise.  If  the  liquid  be 
carried  on  a  spoon  to  the  fauces,  the  déglutition  takès  place  in  a 
more  complète  manner,  but  it  brings  on  a  state  of  extrême  anxiety. 
Whatever  maybe  the  modeof  introduction  of  the  alimentary  substance, 
its  eiitrance  into  the  œsophagus  seems  to  take  place  with  extrême 
slowness,  and  some  minutes  after,  noisy  pharyngeal  movements, 
provoked  by  liquid  remaining  at  its  upper  orifice,  are  still  remarked. 
The  fluids  never  return  towards  the  nasal  fosscc,  and,  moreover, 
direct  examination  of  the  vélum  palati  shows  that  it  is  symmetrical, 
and  that  it  lias  retained  the  perfect  freedom  of  its  normal  move- 
ments. 

XJp  to  the  last  few  days  the  patient  could  still  be  got  out  of  bed, 
and  she  passed  the  hours  of  daytime  seated  in  an  arm-chair.     But 


SYMPTOMS.  357 

thé  symptoms  having  become  suddenly  worse,  she  is  uow  completely 
confined  to  bed. 

Motor  impotence,  complète  in  thc  left  upper  limb,  is  a  little  less 
marked  in  tlie  left.  This  paralysis  is  accompanied  by  a  certain 
degree  of  contracture  ;  tlie  fingers  are  flexed  on  tlie  palm  ;  the  wrist 
is  in  pronation  ;  the  half-flexed  elbow  resists  when  one  tries  to 
straighten  it.  The  muscular  masses  are  wasted,  and  stirred  with 
fibrillary  movements.  The  atrophy,  which  is  more  marked  on  the 
left  than  on  the  right,  is  probably  more  advanced  at  the  root  of  the 
limb  than  at  its  extremity.  Whilst  the  muscles  of  the  shoulder, 
particularly  the  deltoid,  hâve  nearly  disappeared,  leaving  the  bony 
projections  bare,  the  thenar  and  hypothenar  eminences^  though 
lessened,  hâve  still  preserved  a  considérable  thickness. 

In  the  thorax,  the  pectorales  majores  are  affected  in  the  same 
degree  as  the  deltoid  muscles  ;  the  least  touch  brings  up  fibrillary 
motion,  when  it  does  not  show  itself  spoutaneously. 

The  lower  limbs,  which  are  much  less  deeply  affected,  are  equal 
in  size.  They  présent  a  noticeable  emaciation  over  the  whole 
limb  ;  no  group  of  muscles  seems  more  specially  taken  than  the 
others.  They  can  exécute  some  movements  on  the  bed-level.  The 
muscular  masses,  those  of  the  calves  especially,  are  the  seat  of 
abundant  fibrillary  contractions.  Earadaic  examination  of  the 
muscles  enables  us  to  state  that  they  ail  contract  under  the 
influence  of  electricity,  though  the  lower  limbs  react  with  greater 
force  than  the  upper.  The  orbicularis  oris  in  particular  seems 
very  sensitive  to  electric  excitation.  But  muscular  contraction 
does  not  everywhere  take  place  with  its  normal  character,  and,  in 
many  muscles,  it  assumes  the  form  of  fibrillary  motion. 

Sensibility  seems  to  be  preserved  in  ail  its  modes.  The  puise  is 
104.     Kespiration  regular. 

ist  October. — P.  100,     Commencement  of  bed-sore. 

2nd. — P.  108;  11.  26. 

6th. — P.  100;  Pt.  20. 

7th. — P.  120. 

loth. — P.  130.  Extremities  cold.  Urine  turbid,  containing 
neither  sugar  nor  albumen.     Rétention  of  urine. 

I3th.— P.  124. 

I4th. — P.  120. 

23rd. — Debility  has  made  considérable  progress.  The  patient 
bas  scarcely  strength  to  cry  out.     Peeding  has  become  impossible. 


358       LATERAL    SYMMBTKICAL    AMYOTEOPHIC    SOLEEOSIS. 

Extremities  cokl.  Puise  imperceptible.  The  bed-sore  lias  spread' 
over  a  great  brcadtli. 

25th, — Deatli. 

Necropsy  :  Slate  qf  viscera. — The  Jieart  is  small;  no  valvular 
lésions  exist;  the  walls  hâve  their  normal  thickness  and  colour. 
JSfo  lésions  in  the  hmgs.  The  liver,  normal  in  size^  exhibits  no 
cicatrices  ;  same  thiiig  as  regards  spleen  and  kidneys.  The  vesical 
mucous  membrane  is  red,  covered  witli  mammillated  projections, 
lined  witli  purulent  exudation. 

State  of  muscles. — The  muscles  of  the  face  are  very  slender^  but 
their  colour  îs  perceptibly  near  the  normal  hue.  The  masseter,  red 
on  the  surface,  is  yellowish  within.  The  sterno-mastoid,  scalene,  and 
trapezius  muscles  are  well  nourished,  and  présent  a  fine  red  colour. 

The  pectorales  and  the  muscles  of  the  left  upper  limb  are  yellow, 
discoloured,,  thinned,  and  their  appearance  strikingly  contrasts  with 
tliat  of  the  muscles  of  the  neck  ;  the  deltoid  is  especially  altered. 
In  the  hand,  the  muscles  of  the  thenar  and  hypothenar  eminences 
are  discoloured.  The  serratus  magnus,  like  the  pectoralis  major,  is 
pale  and  wasted.  It  is  the  same  thing,  but  to  a  less  degree,  as 
regards  the  abdominal  muscles.  The  diaphragm  has  preserved  its 
normal  colour,  consistence,  and  thickness. 

In  the  lower  extremities,  the  muscles,  although  slender  are 
scarcely  discoloured — a  certain  uumber  of  them  hâve  been  examined. 
The  sartorius,  the  rectus  femoris,  for  the  thigh — in  the  leg,  the 
gemelli,  the  tibialis  anticus,  the  extensor  communis  digitorum — but 
none  of  them  presented  even  that  dead-leaf  colour  which  long  con- 
finement in  bed  so  frequently  gives  to  muscles. 

State  of  nerve  centres. — The  hrain,  the  cerebellum,  and  the  isthmus 
of  the  encephalon  do  not  présent  any  perceptible  altération  ;  the 
arteries  of  the  base  are  healthy.  The  bulbus  rachidicus  présents 
ail  the  signs  of  the  normal  state.  The  tissue  of  the  cord  is  firm  in 
consistence  throughout  ;  there  is  no  évident  atrophy  affecting  the 
différent  columns  of  the  organ.  The  originating  filaments  of  the 
lulbar  nerves,  situated  below  the  facial,  namely,  the  hypoglossus,, 
glosso-pharyngeal,  pneumogastric  and  spinal,  contrast,  by  their  fine- 
ness  and  their  grcy  colour^  with  the  nerve-roots  situated  above  ;  the 
facial  in  particular  is  free  from  ail  altération.  This  extrême  tenuity 
and  this  grey  tint  are  again  met  with  in  a  certain  number  of  the 
anterior  roots  of  the  cord. 

Histologie  studj  :     Muscles. — Examination  of  the  muscles  of  the 


AUTOPSY.  359 

tongue,  several  tiiiies  repeated^  lias  always  giveii  an  almost  négative 
resLilfc.  At  ieast,  we  bave  never  fouud  that  granular  condition  of 
the  muscular  fibre,  nor  that  abundant  nuclei  prolifération  wliich 
characterises  atrophie  degeneration  of  the  muscles,  arrived  at  an 
advanced  degree  of  évolution.  In  the  muscles  of  ihe  face,  on  the 
contrary,  nunierous  fibres  had  lost  their  cross  striation,  and  presented 
a  very  marked  granular  condition  of  the  contents  of  the  sheath. 

In  the  muscles  of  the  npper  limbs  which,  to  the  naked  eye, 
exhibited  a  ycUowcolour  and  a  very  évident  diminution  insize,  micro- 
scopic  examination  revealed  the  présence  of  a  large  number  of  degen- 
erated  primary  bundles.  In  the  thenar  and  hypothenar  eminences, 
particularly  the  fibres  had  undergone  very  marked  simple  atrophy; 
in  other  places,  they  had  largely  lost  their  cross  striation,  and  the 
nuclei  of  the  interstitial  connective  tissue  were  extremely  multiplied. 
On  certain  préparations,  examined  in  glycérine  afterthe  addition  of 
acetic  acid,  we  could  see  the  contents  of  broken  sheaths,  forming 
islets  arranged  in  parallel  séries,  separated  from  each  other,  and 
partly  masked  by  clusters  of  nuclei.  The  muscles  of  the  irun/c  and 
lozoer  Ihnhs  presented  the  same  altération,  but,  especially  the  latter, 
to  a  much  less  advanced  degree. 

Nerves. — The  originating  filaments  of  most  of  the  bulbar  nerves 
bave  been  examined,  and  ail  exhibited  histologie  characters  closely 
akin  to  the  normal  state.  We  could  barely  distinguish  a  few 
fibres  with  granular  contents,  whilst  some  others,  deprived  of  their 
medullary  cylinder,  were  reduced  to  their  sheaths  and  covered  with 
more  numerous  nuclei  than  usual.  The  trunks  of  thèse  nerves  were 
not,  any  more  than  their  roots,  notably  altered  in  their  ulterior 
course.  In  particular,  the  integrity  of  the  fibres  of  the  hypoglossus 
at  the  base  of  the  tongue  hâve  been  observed  ;  the  same  holds 
good  as  regards  the  spinal,  pne?fniof/astric,  and/acial  nerves. 

The  anterïor  roots  of  the  rachidian  nerves,  examined  on  a  level 
with  the  cervical  enlargement,  showed  some  degenerated  fibres  in 
the  midst  of  a  large  number  of  healthy  fibres. 

The  left  médian  nerve  examined,  after  being  hardened,  in  trans- 
verse sections  was  fouud  healthy. 

Nerve  centres. — Préparations  made  after  hardening  in  chromic 
acid  and  coloured  by  carminé  : 

Bulhis  rachidicus. — Examination  of  transverse  sections  of  this 
organ,  taken  at  différent  heights,  enables  us  to  discern  lésions  of 
the  white  and  grey  substances. 


360      LATERAL    SYMMETRICAL    AMYOTROPHIC    SCLEROSIS. 

1°.  Grcy  suJjstance. — The  orîgin-nuclei  qf  ihe  hulhar  ncrvcs  are 
tlie  seat  of  altération  hère.  The  latter,  which  is  essentially  charac- 
terised  by  pigmentary  altération  and  consécutive  atrophy  of  the 
nerve-cells  which  enter  into  the  composition  of  thèse  nuclei,  is 
especially  marked  in  that  of  the  hypoglossus  nerve.  Besidc  some 
cells  which  hâve  remained  healthy,  we  can  see  in  the  others  the 
characters  of  the  lésion  at  ail  stages  of  its  development.  Most 
of  them,  already  invaded  by  yellow  degeneration^  refractory  to  the 
action  of  carminé,  and  notably  diminished  in  volume,  hâve  assumed 
a  globular  form.  They  give  birth  to  rare  prolongations,  pale  and 
thin,  which  it  is  impossible  to  follow,  as  in  the  normal  state,  for  a 
certain  distance  from  their  point  of  origin. 

The  neuroglia  does  not  appear  to  take  any  part  in  the  morbid 
process,  it  has  preserved  its  normal  transparency,  and  it  is  im- 
possible to  discover  any  évident  augmentation  in  the  number  of  its 
nuclei. 

The  cell  groups,  belouging  to  the  several  other  nerves  of  the 
région,  are  less  severely  smitten.  The  cells  are  hère  in  considérable 
number,  and  if  some  appear  to  hâve  undergone  a  decrease  in  size, 
we  find  but  very  rare  examples  of  that  pigmentary  invasion  which 
is  so  distinct  in  the  hypoglossus-nucleus. 

The  olïvary  loclies  are  normal  in  ail  the  sections. 

2°.  WJàte  SîiJjstance. — The  lésion  of  the  white  substance  hère 
occupies  the  whole  extent  of  the  anteriûr  pyramids,  which  are  the 
seat  of  very  manifest  sclerosis,  and  are  vividly  coloured  by  carminé. 
It  may  be  traced,  in  the  fasciclcs,  from  the  point  where  they  émerge 
from  the  protuberantia  to  a  level  with  their  decussation.  Itis  easy, 
on  the  same  sections,  to  perceive  the  perfect  integrity  of  the  nerve 
roots  in  their  intra-bulbar  course.  Itis  especially  very  évident  as 
regards  those  of  the  hypoglossus,  and  contrasts  in  a  striking  manner 
with  the  very  marked  atrophy  of  their  origin-nucleus. 

The  decussation-region  possesses  particular  interest  ;  whilst,  in  the 
anterior  part,  what  remains  of  the  pyramid  stands  out  distinctly 
under  the  form  of  a  transverse  red  band,  we  see  the  sclerosis  advance, 
like  a  wedge,  the  broad  end  of  which  is  behind,  into  the  decussation- 
region,  and  proceed  to  invade,  passing  from  the  opposite  side,  the 
reticulatcd  formation  and  the  superior  part  of  the  latéral  columns. 
The  anterior  cornua  which,  at  this  level,  are  representcd  by  two 
islets  of  grey  substance  completely  isolated  from  the  central  sub- 
stance, contain  a  notable  proportion  of  dcgenerated  cells. 


LESIONS  OP  THE  SPINAL  CORD.  361 

Corel. — ïlie  cord  is  the  seat  of  vcry  extensive  altérations  wliicli 
bear  both  on  the  anterior  cornua  of  the  grey  substance^  and  on  the 
antero -latéral  columns.  It  is_,  also,  to  be  remarked  that,  atleast,  in 
the  cervical  région^  the  lésions  appear  to  hâve  reached  a  more 
advanced  period  of  their  évolution  in  the  left  than  in  the  right 
side  of  the  organ,  which  has  consequently  become  unsymmetrical 
(Plates  IV  and  V). 

Anfero -latéral  cohmns. — Thèse  présent^  on  transverse  sec- 
tions of  the  cord,  ail  the  characters  of  sclerosis  of  the  wliite  sub- 
stance. The  great  connective  tracts,  which  extend  from  the  peri- 
phery  of  the  organ  to  the  grey  substance,  are  thickened,  The 
meshes  of  the  reticulum,  considerably  broadened^  exhibit  numerous 
nuclei.  They  bound  very  unequal  spaces,  in  which  sections  of  the 
axis-cylinder  are  seen.  The  latter  are  mostly  more  slender  than  in 
the  normal  state  ;  in  some  places,  and  on  the  contrary,  they  appear 
hypertrophied.  The  altered  régions  are  vividly  coloured  by  car- 
mine. 

If  we  study  the  distribution  of  this  sclerosis,  it  is  seen  to 
occupy,  throughout  the  whole  leugth  of  the  cord,  symmetrical 
points  in  each  of  the .  halves  of  this  organ.  It  also  recalis  by  its 
mode  of  distribution  the  descending  degenerations,  consécutive 
on  certain  circumscribed  lésions  [en  foyer)  of  the  encephalon,  though 
it  differs  in  certain  particulars. 

In  the  entire  cervical  région,  it  occupies,  in  the  iunermost  part 
of  the  anterior  columns,  a  sort  of  triangle,  the  base  of  which  rests 
on  the  white  commissure  ;  one  of  the  sides  of  the  triangle  borders 
the  anterior  sulcus,  whiist  its  apex  tapers  to  an  end  towards  the 
middle  part  of  this  sulcus.  This  triangle,  broader  on  the  right 
than  on  the  left,  is  seen  no  more  towards  the  lower  part  of  this  région. 
In  the  latéral  columns,  commencing  in  front  at  the  outer  angle 
of  the  anterior  cornu,  it  follows,  within  and  behind,  the  contour  of 
the  grey  substance  without  pcnetrating  its  interior  j  whiist,  on  the 
outside,  it  is  separated  from  the  periphery  by  a  narrow  band  of 
healthy  tissue. 

The  superior  portion  of  the  région,  that  which  is  situated  imme- 
diately  below  the  collar  of  the  bulbus,  diverges  a  little  from  this 
description.  Hère,  in  fact,  the  anterior  cornu  is  surrounded  on  ail 
sides  by  a  sort  of  crowii  of  sclerosed  tissue.  If,  from  the  upper 
portion,  we  descend  towards  the  dorsal  and  lumbar  régions,  we  see 
the  sclerosis  leave  the  anterior  column  and  progressively  diminish  in 


362       LA'I'EUAL    SYMMETEICAL    AMYOTROPHIC    SGLEROSIS. 

extent  in  tlie  latéral  column.  In  tlie  dorsal  région,  the  périphérie 
circlc  of  hcalthy  tissue  enlarges  notably,  whilst  the  sclerosis  leaves 
the  conioui-  of  the  anterior  cornu.  In  the  lumbar  région,  it  has 
gone  oll'  froni  the  posterior  cornu  and  forras  a  sort  of  islet  situated 
in  the  posterior  part  of  the  column,  and  surrounded  on  ail  sides 
by  normal  tissue,  except  behind,  where  it  sends  a  prolongation 
towards  the  pcriphery  and  the  entrance  point  of  the  posterior  roots. 
Ail  the  rest  of  the  white  substance,  and  particularly  the  posterior 
columns,  is  exempt  from  altérations.  The  same  thing  holds  good 
for  the  anterior  roots  in  tlieir  intra-spinal  course. 

Grey  substance. — Hère,  exactly  limited  to  the  area  of  the  cornua 
of  grey  substance,  and  symmetrically  disposed  in  the  two  halves  of 
the  cord,  we  again  meet  with  the  cellular  lésion  whicli  has  been 
described  in  référence  to  the  nucleus  of  the  hypoglossus.  Striking 
indiscriminately  and  at  hazard,  as  it  were,  différent  groups  of 
thèse  cornua,  it  gradually  diminishes  in  extent,  in  pro])ortion  as  it 
approaches  the  inferior  région  of  the  cord.  Whilst,  at  the  cervical 
enlargement,  we  can  hardly  compute  the  number  of  cells  spared  at 
one  fifth  of  the  total  number,  in  the  lumbar  région  more  thana  half 
bave  j)reserved  the  characters  of  the  normal  state.  The  vesicular 
column  of  Clarke  has  not  been  spared  ;  but  ail  the  éléments  of  the 
posterior  cornua  hâve  escajied  degeneration. 

The  neuroylïa  has  not  hère,  any  more  than  in  the  bulbus,  taken 
an  active  part  in  the  morbid  work  ;  and,  in  ail  the  sections,  we  may 
see  cclls  reduccd  to  a  few  pigmentary  granulations  in  the  midst  of  a 
perfectly  nornud  tissue.  However,  the  grey  substance  has,  in  cer- 
tain spots,  been  disorganised  in  its  entirety,  and  we  can  observe,  in 
the  upper  région  of  the  cord,  the  présence  of  genuine  foci.  Ver- 
tically  elongated,  they  occupy  symmetrically  the  two  anterior  cornua, 
the  limits  of  which  they  do  not  cxceed.  The  sections  which  were 
made  across  their  middle  portion  show  only  a  thick  mass  of  tissue 
becoming  strongly  coloured  by  carminé,  projecting  above  the  sur- 
face of  the  section,  in  which  it  is  difficult  to  distinguish  any  élé- 
ment. But  thèse  foci,  swollen  in  their  middle  part,  taper  off  at 
both  ends,  and  it  is  in  thèse  jjoints  we  should  examine  tliem.  We 
then  see  that  they  begin  by  a  certain  Jiumber  of  little  rounded  islets, 
tho  tissue  on  a  level  with  which  is  evidently  thickened  and  rendered 
less  transparent  without  any  manifest  multiplication  of  neuroglia- 
nuclei  being  remarked. 


NOTE    ON    A    CASE    OF    GLOSSO-LAllYNGEAL    PARALYSIS, 
EOLLOWED  BY  AUÏOPSY.     Bï  J.  M.  Chakcot. 

(Sce  Lecture  XIII,  p.  192.) 

By  the  général  aspect  of  its  symptoms,  tlie  case^  whicli  I  am 
going  to  describe  in  full  détail,  belongs  to  the  clinical  type  created 
by  Ur.  Duchenne  (de  Boulogne),  under  tlie  name  of  progressive 
muscular  paralysis  of  the  tongue,  of  the  vélum  palati,  and  of  the 
lips  ;  but,  considered  anatomo-pathologically,  it  differs  remarkably 
from  ail  cases  of  the  same  kind  which  hâve  been  published  up  ta 
the  présent.  It  is  on  this  account,  chiefly,  that  it  lias  seemed  to 
me  worthy  of  attracting  the  attention  of  the  reader  fur  an  instant. 

Case. — Baj —  Marie-Françoise,  agcd  68,  was  first  admitted  to 
the  General  Lifirraary  of  the  liospice  de  la  Salpêtrière  April  ii, 
1869,  to  be  treated  for  a  slight  bronchitis;  it  was  not  remarked  at 
that  time  that  her  speech  was  embarrassed.  However,  her  children 
affirm  that  they  had  remarked  that,  duriug  the  past  year,  she  ex- 
pressed  herself  with  great  difiiculty  from  timc  to  time.  About  last 
May  déglutition  difficulties  appear  to  hâve  begun.  It  is  certain  that, 
since  that  period,  it  often  happened  that,  in  swallowing,  food  went 
the  wrong  way,  and  that  the  [)aticnt  was  taken  with  violent  lits  of 
coughing.  During  meals,  she  very  often,  also,  threw  up  food 
through  the  nasal  passages.  An  exaspération  of  ail  the  symptoms 
appears  to  hâve  been  suddenly  ])roduced,  about  a  month  before  her 
second  admission  into  the  infirmary,  which  took  place  on  the  ]  oth 
September.  In  the  space  of  a  few  days  the  articulation  of  words 
seems  to  hâve  become  alniost  impossible,  and,  from  this  moment, 
the  difhculty  in  swallowing  food  and  drink  appears  to  hâve  grown 
rapidly  worse.  The  patient  déclares  that  this  abrupt  aggravation 
was  not  accompanied  by  giddiness  or  any  other  phenomena  of  the 
same  kind.     The  weakness  in  the  voluntary  movements  which  at 


364  GLOSSO-LAEYNGEAL   PARALYSIS. 

présent  exist  iu  tlie  Icft  upper  limbj  to  whicli  référence  will  again 
be  made,  dates  back  four  montlis_,  and  was  produced  slowly,  in  a 
progressive  manner. 

Présent  state,  September  lo,  1869. — The  articulation  of  words 
is  already  so  much  embarrassed  that  the  patient  cannot  succeed  in 
making  lierself  compréhensible  ;  ail  the  attempts  at  spcaking  which 
she  makes  resuit,  in  fact,  in  the  production  of  a  hollow  grunting, 
with  nasal  twang,  However,  as  well  as  can  be  determined  in  the  case 
of  a  patient  who  can  only  express  herself  by  signs,  lier  intelligence 
seems  perfectly  preserved.  The  tongue  is  not  so  iuert  as  one  might 
believe  from  wliat  has  been  said  ;  it  has  retained  its  normal  shape, 
thicknesSj  and  dimensions  ;  its  surface  offers  no  abnormal  wrinkle 
or  furrow;  liowcver,  on  examiuing  its  border  with  the  greatest 
care,  some  slight  fibrillary  movements  seem,  from  time  to  time,  to 
be  perceptible.  B —  can  still  protrude  it  easily  enough,  and  move 
it  from  right  to  left,  but  she  cannot  either  turn  up  the  point,  nor 
apply  its  dorsal  surface  to  the  palate. 

The  movements  of  the  orbicularis  oris  are  very  markedly  weak- 
ened.  The  patient  cannot  simulate  the  act  of  kissing  or  whistling, 
but  she  can,  by  exerting  ail  her  strength,  blow  out  a  candie 
distant  10  centimètres  or  more  from  her  mouth. 

The  difHculty  of  déglutition  is  most  marked.  Wlien  B —  'iyishes 
to  swallow  a  fiuid,  she  first  rejects,  almost  always  voluntarily,  a 
considérable  quantity  from  her  mouth.  Then,  putting  the  thumb 
of  the  right  hand  on  one  side  of  the  larynx,  she  seems  to  désire  to 
assist  the  upward  movement  of  this  organ,  which  soon  occurs; 
but,  hardly  has  the  first  stage  of  déglutition  taken  place  than  a 
state  of  extrême  anxiety  supervenes  ;  for  over  five  minutes  the 
patient  seems  threatened  with  suffocation  ;  she  does  not  generally 
cough,  but  at  every  inspiration  a  sonorous  laryngeal  sound  is  heard 
resembling,  to  a  certain  extent,  what  is  observed  in  certain  cases  of 
cedema  glottidis.  It  often  happens  that  some  drops  of  the  fluid 
taken  in  are  returned  through  the  nose.  The  déglutition  of  solid 
food,  or  better  still,  semi-Hquid  food,  is,  perhaps,  less  difïicult  than 
that  of  ûuids  ])roper,  but  it  is  still  generally  troubled  by  the  same 
syraptoms. 

Direct  examination  of  the  vélum  palati  does  not  detect  any 
deformity  ;  the  uvula  occupies  the  mesial  line,  and  does  not  hang 
down  too  much  ;  the  membranous  vélum  nppears  likcwise  to  con- 
•tract  in  a  nearly  normal  manner  when  titillated. 


SYMPTOMS.  365- 

A  tliick  and  viscid  saliva  gathers  constantly  in  lier  mouth  and 
sometimes  runs  out.  The  patient  is  often  found  using  her  fmgers 
to  clear  her  mouth  of  the  thick  mucus  and  portions  of  food  which 
hâve  accumulated  there.  On  account  of  the  difficulty  of  déglutition 
the  process  of  feeding  takes  place  in  a  very  incomplète  manner  ; 
the  patient  shows,  at  every  instant,  by  significant  signs,  how  dis- 
tressing  it  is  for  her  to  be  unable  to  satisfy  her  hungcr. 

She  is  very  thin,  and  is  already  much  weakened.  On  examining 
the  state  of  the  muscular  System  in  the  différent  parts  of  the  body, 
we  remark  what  follows  : — The  muscles  of  the  left  shoulder  are 
manifestly  more  emaciated  than  those  of  the  right;  besides,  the 
deltoid  is  almost  constantly  stirred  by  very  marked  fibrillary 
movements,  which  are  spontaneously  produced  or  which  are  readily 
aroused  by  a  slight  touch  when  they  cease  to  occur.  Owing  to 
the  debility  of  thèse  muscles,  the  patient  finds  it  diflficult  to  raise 
her  arm,  and  she  cannot  lift  her  left  hand  to  her  mouth.  The 
arm  and  forearm,  on  this  side,  are  not  more  emaciated  than  the 
corresponding  parts  of  the  right  upper  extremity  ;  their  muscular 
masses,  however,  are  hère  and  there  the  seat  of  some  fibrillary 
contractions.  Finally,  movements  of  préhension  are  accomplished 
with  the  left  hand  as  well  as  with  the  right,  and  there  is  no  trace  of 
prédominant  atrophy  iu  the  muscles  of  the  thenar  and  hypothenar 
eminences. 

The  right  upper  extremity  is  uniformly  emaciated  throughout  ;  no 
partial  atrophy  exists  anywliere.  However,  fibrillary  movements, 
but  slightly  marked  in  truth,  occur  in  some  parts,  chiefly  at  the 
shoulder. 

The  lower  extremities  are  both  emaciated  to  the  same  degree  ; 
there  is  no  différence  in  this  respect  between  the  right  and  left 
siues.  Their  movements  are  normal,  but  notably  weakened.  B — , 
however,  can  stand,  and  take  some  steps  in  the  ward,  but  not 
without  great  fatigue.  Ou  the  left,  the  muscles  of  the  anterior 
part  of  the  thigh  and  those  of  the  calf  are  the  seat  of  fibrillary 
contractions. 

Eibrillary  contractions  are  likewise  observed  in  the  cervical  por- 
tion of  the  trapezius,  and  in  the  sterno-cleido-mastoidei.  Never- 
theless,  the  action  of  the  muscles  which  move  the  head  is  of  suffi- 
cient  strength,  and  the  attitude  of  the  latter  is  quite  normal. 

No  visual  disturbance  appears  to  exist;  the  pupils  are  of  the 
same  diameter.    Sensory  dérangements  are  nowhere  tp  be  discerned.^ 


366  GLOSSO-LAEYNGEAL  TARALYSIS. 

The  puise  is  weak,  but  not  quickened  ;  the  température  of  tlie  body 
is  normal.  Neither  sugar  nor  albumen  was  found  in  ilie  urine, 
althougli  it  was  frequently  examined. 

October  35th. — Debility  bas  raade  vast  progress.  B —  can  no 
longer  sustain  lierself  on  her  legs.  Yesterday,  she  fell  wlien  gettiug 
eut  of  bed^  and  was  unable  to  rise  without  help.  Déglutition  lias 
become  absolutely  impossible,  and,  for  some  days  past,  recourse  was 
liad  to  tbe  stomacli-pump.  It  is  again  remarked  that  tbe  move- 
raents  of  the  vélum  palati  take  place  tolerably  well  under  the  influ- 
ence of  direct  stimulation.  It  is  also  remarked  that  the  toiigue 
can  still  be  protruded  beyond  the  lips,  and  slightly  turned  from 
right  to  left,  but  its  movements  are  evidently  slower  and  weaker 
than  in  the  past.  Its  size,  however,  bas  not  been  notably  dimi- 
nished  ;  its  dorsal  surface  is  still  perfectly  smooth,  and  no  capillary 
contractions  are  observed.  Its  borders  alone,  are,  in  certain  poiuts, 
plaited,  wrinkled,  and  exhibit  almost  incessant  vermicular  move- 
ments. 

26th. — Eor  the  firsttime,  it  is  observed  that  the  puise  is  fréquent, 
— 130.     However,  the  température  of  the  rectum  is  37'4'°  C. 

27th. — The  puise  is  still  quicker  than  onyesterday.  The  num- 
bcr  of  its  pulsations  rises  probably  to  150  per  minute.  It  is  very 
small,  almost  imperceptible.  The  respiration  isat  32.  The  inspi- 
rations are  very  distressing,  and  accompanied  by  strong  contraction 
of  the  sternp-cleido-mastoidei  and  scalcni.  There  is  extrême 
anxiety.  When  we  ask  the  patient  if  she  is  sufPering,  she  puts  her 
hands  over  the  precordial  région,  and  gives  us  to  understand  that 
she  there  expériences  a  suffering  which  she  cannot  define.  By  pal- 
patiou,  and  percussion,  it  is  ascertained  thst  the  pulsations  of  the 
lieart  are  sufficiently  strong.  The  second  sound  is,  at  the  base, 
hardly  distinct  ;  it  is,  on  the  contrary,  tolerably  wcU  marked  at  the 
apex.     Wo  abnormal  sounds  are  lieard. 

aSth. — Puise  128  ;  temp.  rcct.  37*6°  (C.)  ;  rcsp.  28.  The  inspira- 
tions bave  become  more  and  more  distressing,  and  are  accompanied 
by  energetic  contractions  of  the  sterno-mastoidei,  scaleni,  pectorales 
majores,  and  of  the  anterior  border  of  the  trapezius.  It  is  remarked 
that  the  belly  subsides  in  the  epigastric  région  when  the  ribs  and 
claviclcs  rise.     Hence  there  is  inertia  of  the  dinphragm. 

29th. — Same  conditionas  yesterday.  The  puise  is  extremely  rnpid ; 
temp.  rect.  37"6°.  The  patient  refused  to  permit  the  stoinach- 
pump  to  be  introduced.     In  the  evening  :  extrême  dyspnœa;  32 


AUTOPST.  367 

respirations  ;  tliere  were  probably  more  than  150  pulsations  per 
minute;  thc  rectal  température  is  37*6°  C.  The  patient  suddenly 
succumbs  in  the  night,  without  a  struggle. 

Necroscopy,  made  twenty-four  hours  after  deatli. — Cadaveric 
rigidity  is  well  marked  every  where. 

A.  a.  State  of  viscera. — The  heart  is  normal  in  size  ;  the  right 
ventricle  is  distended  by  black  clots.  No  lésion  of  thc  valves  exists  ; 
the  muscular  walls  of  the  left  ventricle  are  perhaps  a  little  pale,  but 
rather  firm.  The  lungs  are  very  emphysematous,  especially  the 
right  ;  they  do  not  exhibit  any  other  altérations.  The  liver  is  of 
normal  size.  The  supra-renal  capsules,  kidneys,  and  spleen  are 
healthy.  The  stomach  and  intestines  are  shrivelled  and  shrunken  ; 
otherwise  they  présent  no  perceptible  altération. 

/3.  State  of  tmiscles. — The  extrinsic  muscles  of  the  tongue  and 
of  the  supra-  and  sub-hyoid  régions  exhibit  a  fine  red  colour  ;  per 
contra,  the  proper  muscles  of  the  tongue  are  distinguished  by  their 
pallor  and  by  an  évident  diminution  in  firmness. 

In  the  larynx  ail  the  intrinsic  muscles  appear  to  be  healthy,  with 
the  exception  of  the  arytenoidei,  the  posterior  crico-arytenoidei,  and 
the  crico-thyroidei,  which  are  manifestly  atrophied,  and,  hère  and 
there,  show  a  very  évident  yellow  hue.  The  crico-arytenoidei  and 
the  crico-thyroidei  of  the  left  side  are  besides  much  more  altered 
than  their  congenerous  muscles,  and  they  are  noticed  to  havc  little 
ecchymotic  spots  in  the  vicinity  of  their  insertion. 

The  muscles  oî  the  p/iarpix  do  not  seem  to  hâve  undergone  any 
perceptible  altération.  The  muscular  wall  of  the  œsophagus  appears 
to  be  normal  in  size  and  consistence.  Both  sterno-cleido-mastoidei 
are  slender,  but  red. 

The  trapezius  hasayellovi?  tint  throughout;  this  nbnormal  colour 
is  especially  marked  at  the  left  anterior  border  of  the  cervical  por- 
tion of  this  muscle.  In  this  spot,  the  muscular  fibres  are  very  pale, 
very  friable,  and  separated  by  little  lumps  of  fat. 

The  same  altération  is  remarked  in  the  anterior  portion  of  the 
deltoid  on  the  left  side.  The  posterior  part  of  the  same  muscle  is 
rclativcly  little  altered.  The  right  deltoid  présents  a  fine  red 
colour. 

The  two  pectorales  are  slender,  but  nowise  discoloured;  the 
intercostales,  on  the  contrary,  are  atrophied  and  yellow. 

In  the  arms,  forearms,  and  hands,  the  muscles,  alike  in  left  and 
right  side,  présent  the  appearance  of  the  normal  state.  The  diaphragm 


3G8  GLOSSO-LAEYNGEAL    TAIiALYSIS. 

does  not  show  any  perceptible  altération.  Some  muscles  of  the 
lowcr  extrcmities  hâve  been  examinée!  ;  they  présent  tlie  normal 
characters,  so  far  as  colour  and  consistence  are  concerned. 

-y.  State  of  nerve-centres  and  of  hilhar  nerves. — The  brain  proper 
and  the  différent  parts  of  the  isthmus  do  not  eshibit  any  perceptible 
altération  ;  the  bulbus  in  particular  and  the  protuberantia  offer  ail 
the  appearances  of  the  normal  state.  No  trace  of  atrophy  or  indu- 
ration is  detected.  The  arteries  of  the  base  are  barely  atheromatous. 
The  cord,  examined  externally  and  in  sections  taken  at  différent 
lieights,  appears  also  to  be  perfectly  healthy. 

The  root-filaments  of  a  certain  number  of  bulbar  nerves,  namely, 
the  hypoglossusj  the  pneumogastric,  the  glosso  pharyngeus,  and  the 
spinal,  especially,  are  slender.  As  to  the  nerve-truuks,  arising 
from  thèse  roots,  they  appear  to  be  a  little  less  in  size  than  in  the 
normal  state,  but  they  hâve  not  undergone  any  change  in  colour. 

B.  Histologie  study  :  a.  Muscles. — Towards  the  tip  of  the  tongue, 
w'here  the  muscular  fibres  were  paler,  perhaps  one  half  of  the 
primary  fibres  presented  throughout,  with  no  well-marked  réduc- 
tion in  size,  a  certain  degree  of  granular  altération,  with  or  without 
the  disappearance  of  the  cross  striée. 

Next  we  find,  by  means  of  préparations  coloured  with  carminé,  a 
very  évident  multiplication  of  sarcolemma-nuclei  on  a  great  number 
of  primary  fibres.  The  connective  tissue,  interposed  between  thèse 
bundles  exhibits  nearly  everywhere  a  greater  number  of  nuclei  than 
in  the  normal  state. 

It  is  remarkable  that  the  prolifération  of  the  nuclei  of  the  sarco- 
lemma  is,  perhaps,  more  manifest  on  the  sheaths  of  the  bundles 
which  havc  preserved  the  cross  striœ,  and  wliich  are  only  afî'ected 
in  a  very  mild  degree,  by  granular  degeneration,  than  on  the 
bundles  where  this  dégénéra tion  is  most  marked. 

Hère  and  there  some  sarcolemma-sheaths  were  found  void  of 
contractile  substance,  and  filled  by  masses  of  nuclei.  Thèse,  some- 
times,  exhibited  the  hour-glass  form.  Pinally,  on  some  préparations, 
we  saw  sheaths  shrunken  on  themselves,  and  only  enclosing,  in  tlieir 
almost  effaced  cavity,  granulations  of  fatty  ^ispect,  or  clusters  of 

nuclei. 

The  fatty  granular  altération  of  the  primary  bundles,  the  absence 
of  cross  strise,  and  the  prolifération  of  nuclei  of  the  perimysium^ 
and  of  the  sarcolemma,  are  met  with  in  ail  the  other  régions  of  the 
tongue,  but  in  a  less  degree  than  at  the  tip. 


LESIONS    OP    MUSCLES.  369 

Althougli  they  hâve  preserved  their  normal  red  colour,  the  ex- 
trinsic  muscles  of  the  tongue  hère  and  there  show  some  primary 
fascicles  where  we  readily  recognise  the  granular  fatty  altération 
and  the  multiplication  of  the  nuclei  of  the  sarcolemma  or  of  the 
perimysium.  The  same  remark  may  apply  to  the  muscles  of  the 
pharynx,  which,  likewise,  appear  healthy  to  the  naked  eye.  As  to 
the  muscles  of  the  larynx,  those  among  them  which  presented  a 
marked  yellow  colour  to  the  naked  eye,  as  the  posterior  crico- 
arytenoidei,  for  instance,  exhibit  nearly  the  same  degree  of  granulo- 
fatty  degeneration  as  the  tongue. 

The  muscles  of  the  arm  and  forearm,  those  of  the  hand  (thenar 
and  hypothenar  eminences),  although  they  appeared  quite  healthy 
to  the  naked  eye  in  colour  and  consistence,  yet  exhibited,  under 
the  microscope,  a  good  number  of  primary  fibres  which  had  lost 
their  cross  strise,  and  displayed  granulo-fatty  change,  and  mul- 
tiplication of  the  sarcolemma-nuclei  in  a  more  or  less  marked 
manner. 

The  yellow  muscular  fibres,  derived  from  the  anterior  portion  of 
the  trapezius  and  of  the  deltoid  on  the  left,  in  addition  to  the 
granulo-fatty  altération  extending  to  a  very  large  number  of 
primary  fibres,  exhibited  an  accumulation  of  large  fatty  drops 
interposed  between  the  primary  bundles. 

The  muscles  of  the  lower  extremities  (thigh  and  plantar  muscles) 
hâve  been  examined  in  several  parts.  The  granulo-fatty  altéra- 
tion of  some  of  the  primary  bundles  has  been  noticed,  in  a  very 
distinct  manner.  But  the  bundles  thus  altered  were  less  numerous 
there  than  in  the  corresponding  parts  of  the  superior  extremities. 

(3.  Cranial  nerves. — On  ail  the  préparations,  in  the  fresh  state, 
derived  from  the  very  slender  radicular  filaments  of  several  bulbar 
nerves,  from  the  hypoglossus  for  instance,  it  is  remarked,  not 
without  surprise,  that  the  nerve-tubes  hâve  preserved  their  medul- 
lary  cylinders. 

We  did  not  succeed  in  distinctly  detecting  the  existence  of 
empty  and  shrunken  sheaths.  Fine  granulations,  few  in  number, 
however,  are  uniformly  disseminated  over  the  whole  extent-of  some 
nerve-tubes.  Nowhere  are  they  in  masses,  under  the  form  of 
granular  bodies. 

The  trunks  of  the  hypoglossus,  spinal,  and  pneumogastric 
nerves  did  not  présent  any  other  altération  than  this  same  granular 
state  of  some  nerve-tubes.     This  altération  is  very  marked  in  the 

VOL.  II.  34 


3Î'Ô  GLOSSO-LARTNGEAL   PAEALYSIS. 

inferior  laryngeal  nerve.  The  phrenic  and  the  great  cervical  sym- 
patlietic  présent  the  characters  of  the  normal  state. 

7.  Uxamination  ofnerve-centres,  préparations  Jiardened  hy  cJiromic 
acid  and  coloured  hy  carminé.  1°.  Spinal  cord. — Cross  sections  of 
différent  parts  of  the  lumbar  enlargement. 

Examination  of  the  white  columns  shows  neither  diminution 
in  the  diameter  of  the  nerve-tubes,  nor  multiplication  of  the 
neuroglia-nuclei,  nor,  lastly,  any  thickening  of  the  connective 
tracts  which  radiate  from  the  grey  centre  towards  the  periphery  of 
the  cord. 

In  the  grey  substance,  and  more  especially  in  the  area  of  the  ante- 
rior  cornua,  ail  the  altérations  are  concentrated  and,  again,  they 
occupy  only  the  nerve-cells  hère  ;  for  hère,  also.  the  neurogha  is 
normal,  or  only  présents  scarce  visible  traces  of  nuclei  multiplica- 
tion. 

Some  of  the  nerve-cells  hâve  preserved  ail  the  characters  of  the 
normal  state  ,•  they  are  few  in  number,  since  they  stand  for  less 
than  a  third  on  each  préparation.  They  are  readily  recognised  by 
the  following  peculiarities  :  they  are  still  furnished  with  their  pro- 
longations which,  like  the  cell  itself,  are  coloured  vividly  and  uni- 
formly  by  carminé.  The  nucleus  and  nucleolus  are  very  distinct. 
The  small  quantity  of  pigment  which  they  often  contaiu  in  the 
normal  state  is  not  augmented. 

The  cells  which  show  the  first  degree  of  altération  are  imme- 
diately  recognised  by  the  very  intense  yellow  ochreous  hue  which 
they  exhibit  in  the  greater  part  of  their  extent.  This  colour 
results  from  the  présence  of  pigmentary  granules  united  under  the 
form  of  iittle  lumps,  and  not  affected  by  carminé.  The  parts  of  the 
cell  which  hâve  not  been  invaded  by  pigment  become,  on  the  con- 
trary,  coloured,  almost  as  in  the  normal  state.  The  nucleus  and 
nucleolus  are  yet  more  visible  and  more  coloured,  but  the  prolonga- 
tions are  generally  very  short,  as  if  withered,  or  worse  still,  they 
hâve  completely  disappeared.  At  the  same  time,  the  cell  diminishes 
in  size  ;  it  tends  to  lose  its  angular  outhne,  and  acquires  a  globular 
form.     • 

At  a  more  advanced  degree  of  the  morbid  process,  the  cell,  yet 
more  diminished  in  its  dimensions,  absolutely  deprived  of  its  pro- 
longations, is  now  only  represented  by  a  Iittle  mass  of  yellow  granules, 
The  nucleus  and  the  nucleolus  hâve,  in  gênerai,  completely  dis- 
appeared.    There  are,  however,  cases  where  the  latter  still  persists  ; 


LESIONS.  OP   THE    SPINAL   CORÏ).  371 

then  it  is  the  only  portion  of  tlie  cell  which  has  retained  the  pro- 
perty  of  being  coloured  with  carminé. 

Lastlj,  we  find  hère  and  there,  in  points  formerly  occupied  by  a 
cell,  scattered  yellow  disintegrated  granulations.  This,  doubtless,  is 
the  final  term  of  the  altération.  In  such  a  case,  we  no  longer  find 
the  slightest  trace  of  nucleus  or  nucleolus. 

The  altération  of  the  ganglionic  cells  is  uniformly  spread  over  the 
whole  extent  of  the  anterior  cornua  ;  it  does  not  specially  aifect  cer- 
tain groups  of  cells,  so  that  healthy  cells,  and  cells  diseased  in 
différent  degrees,  are  everywhere  intermingled.  The  small  cells  of 
the  posterior  cornua  did  not  appear  to  exhibit  any  trace  of  yellow 
degeneration. 

Comparative  measurements^  taken  with  the  help  of  good  prépa- 
rations obtained  from  the  same  points  in  the  same  région  of  a  healthy 
cord,  hâve  shown  what  foUows  : 

The  cells  which  hâve  retained  the  property  of  being  coloured  by 
carminé  throughout  (healthy  cells),  hâve  the  same  dimensions  as 
the  cells  of  the  normal  préparation  ;  ail  the  cells  which  hâve  lost 
their  prolongations  are  atrophied.  So  long  as  the  nucleolus  is 
visible,  it  préserves  its  normal  size. 

Sections  from,  dorsal  and  cervical  régions. — The  cell-alterations 
are  identical  with  those  in  the  lumbar  région,  but  more  marked, 
especially  in  the  cervical  enlargement.  The  cells  of  the  vesicular 
column  are  altered  to  the  same  degree  as  those  which  constitute 
the  groups  of  the  anterior  cornua. 

2°.  Bulbar  région  :  a.  Section  from  immediately  ahve  the  ca  la- 
mus  point. — The  origin-nucleus  of  the  hypoglossus,  the  lower  part 
of  which  is  visible  at  this  level,  exhibits  very  manifest  altérations, 
which,  hère  also,  bear  exclusively  on  the  nerve-cells  ;  the  neuroglia 
is  intact  ;  its  vessels  perhaps  are  larger  than  in  the  normal  state  ;  in 
any  case,  they  appear  gorged  with  blood-corpuscles. 

The  greater  part  of  the  cells  (about  two  thirds  of  them)  exhibit 
ail  the  grades  of  the  pigmentary  altération,  described  above  in 
référence  to  the  différent  régions  of  the  spinal  cord.  The  altered 
cells  are  disseminated  everywhere  and  mingled  with  healthy  cells  -, 
they  do  not  occupy  any  particular  spot  by  préférence  ;  yet  perhaps 
they  are  more  numerous  than  elsewhere  towards  the  external  limit 
of  the  nucleus. 

Behind  and  external  to  the  hypoglossus-nucleus  we  can  study  the 
originating  cell-groups  of  the  spinal  nerve.     Thèse  cells  hâve  mostly 


373  GLOSSO-LARYNGEAL   PARALYSIS. 

preserved  the  characters  of  the  healthy  state.  A  considérable  num- 
ber  of  them,  however,  hâve  gone  tlirougli  différent  stages  of  pig- 
inentary  altération,  principally  towards  the  external  région  of  the 
nucleus.  It  is  known  that,  in  the  normal  state,  there  exists  in  this 
spot  sorne  more  or  less  piginented  cells^  but  the  number  then  is 
much  more  restricted. 

h.  Section  from  the  middle  of  the  olivary  hodies. — The  cells  of  the 
convolutions  of  the  olive  do  not  exhibit  any  appréciable  altération. 
Those  which  constitate  the  nucleus  of  the  hypoglossus  at  this  level 
are,  on  the  contrary,  very  numerously  affected.  The  origin-cells  of 
the  pneumogastrie  do  not  appear  to  beso  deeply  altered.  Between 
the  nucleus  of  the  hypoglossus  and  that  of  the  pneumogastrie  in 
this  région,  lie  the  small  cell-groups,  which  L.  Clarke  connects 
with  the  nucleus  of  the  facialis.  The  cells  of  this  group  appear 
remarkably  small  and  few  in  number.  They  do  not,  however, 
exhibit  the  pigmentary  altération.  A  section  taken  a  little  above 
the  preceding  one  enables  us  to  state  that  the  origin-cells  of  the 
glosso-pharyngeus  are  not  obviously  altered. 

c.  Section  from  the  most  superior  jportion  of  the  olivary  hodies. — 
This  section,  which  contains  the  nuclei  of  the  facial  and  of  the 
external  oculo-motor,  as  well  as  that  of  the  trigeminus  (?  sensory 
portion)  according  to  Stilling,  shows  that  a  large  number  of  cells 
of  the  f as cic7il7/,s  teres  and  a  small  number  of  cells  of  the  trigeminus 
exhibit  ail  the  characters  of  the  altération  above  described. 

One  of  the  most  interesting  points  of  this  observation  is, 
unquestionably,  the  existence  of  an  altération  which,  in  a  sys- 
tematic  manner,  so  to  say,  occupies  the  nerve-cells,  not  only 
throughout  the  whole  height  of  the  spinal  cord,  but  also  in  the 
bulbus,  from  which  cornes  the  progressive  disorganization  or  even 
the  complète  destruction  of  a  considérable  number  of  thèse 
organic  éléments.  In  the  bulbus,  the  altération  bears  particularly 
on  the  origin-nuclei  of  the  hypoglossus  and  of  the  spinal  nerves  ; 
but,  it  is  also  observed,  though  in  a  lesser  degree,  in  the  nuclei  of 
the  pneumogastrie  and  of  the  facial.  In  the  spinal  cord,  it  is  limited 
to  the  great  nerve-cells  of  the  anterior  cornua,  termed  motor  cells  ; 
the  cells  of  the  posterior  cornua  do  not  appear  to  be  affected.  It 
is  met  with  in  ail  the  régions  of  the  cord,  but  it  certainly  pré- 
dominâtes in  the  cervical  enlargement. 

In  what  does  this  altération   consist?      The  accumulation  of 


SYSTEMATIC   LESION   OF  NERVE-CELLS.  373 

yellow  pigment  seems  to  play  a  large  part  ;  it  appears  to  be  the 
initial  fact.  The  atrophy  of  the  cell-prolongations,  that  of  the 
nucleus,  and  lastly,  that  of  the  nucleolus,  are  consécutive  pheno- 
mena.  Hâve  we  hère  a  process  of  slow  irritation,  or,  on  the  con- 
trary,  of  a  quite  passive  atrophy  ?  Nothing  can  be  decided  with 
respect  to  this  from  a  considération  of  the  anatomical  characters 
merely  ;  but  it  may,  I  believe,  be  af&rmed  that  this  morbid  process, 
whatever  it  be,  affected  primarili/  the  cell  ;  it  was  not  communi- 
cated  to  it  from  without.  In  fact,  the  reticulum  which,  on  ail 
sides,  surrounds  the  diseased  cells,  exhibits  no  other  altération 
than  in  being  more  transparent  than  in  the  normal  state — this 
being  due,  in  ail  likelihood,  to  the  disappearance  of  a  great  number 
of  cell-prolongations  ;  in  cord,  as  in  bulbus,  we  observe  in  it  neither 
foci  of  gramdar  d'mntegraûon,  nor  trace  of  fibrillary  metamor- 
phosis,  nor  even  multiplication  of  myelocites.  Hence,  it  cannot  be 
admitted  that  a  process  of  irritation,  or  even  of  simple  disintegra- 
tion,  should  hâve  first  been  set  up  in  the  connective  web  of  the  grey 
substance,  thence  to  be  propagated  to  the  nerve-elements.  But  one 
might  be  tempted  to  suppose  that  the  starting-point  of  the  altéra- 
tion of  the  ganglionic  cells  should  be  sought  for  external  to  the 
nerve-centres,  that  is  to  say,  in  the  peripheral  nerves.  This  view  is 
not  acceptable  ;  it  is  in  formai  contradiction  with  nnmerous  facts, 
ail  of  which  it  is  needless  to  recall.  We  will  confine  ourselves  to 
pointing  out  that,  according  to  the  researches  of  M.  Vulpian,  com- 
plète section  of  the  peripheral  nerves,  and  of  the  hypoglossus  in 
particular,  bas  no  marked  influence  on  their  origin-cells.  Now,  it 
has  been  seen  that,  in  the  case  which  occupies  us,  the  branches  of 
the  différent  bulbar  nerves  exhibit  very  minute  histologie  altéra- 
tions at  most,  though  the  cell-groups  from  which  they  emanate 
were,  for  the  most  part,  profoundly  diseased.  Whence,  we  think, 
it  is  legitimate  to  conclude  that  the  ganglionic  cells  hâve  been,  in 
bulbus  and  in  cord,  the  primary  seat  of  the  disease,  that  the 
peripheral  nerves  hâve  been  only  secondarily  affected  consecutively 
on  the  lésion  of  the  nerve-centres. 

If  now  the  attention  be  directed  to  the  trophic  lésions  which  are 
presented  by  the  muscular  System  of  the  life  of  relation,  we  shall  be 
struck  by  the  singular  mode  of  distribution  of  thèse  fascicle-lesions 
in  différent  parts  of  the  body.  Manifestly,  we  hâve  not  hère  to  deal 
with  a  common  case  of  progressive  muscular  atrophy  j  the  lésions 
of  the  primary  (ultimate)  bundles  are,  indeed,  those  which  belong 


374  GLOSSO-LABYNGEAL  PAEALYSIS. 

to  the  latter  affection,  and  we  find  hère  sometimes  granulo-fatty 
degeneration,  sometimes  simple  atrophy  of  the  fascicles  with  proli- 
fération of  the  sarcolemma-nuclei.  But  they  are  not,  as  it  were, 
concentrated  on  any  muscle  or  sets  of  muscles  ;  they  are  dissemi- 
nated  a  little  everywhere,  and  we  always  found,  in  the  most  various 
régions,  diseased  fibres  intermingled  amongst  perfectly  healthy 
fibres.  They  were,  however,  more  affected  and  more  common  in 
certain  muscles  than  in  others  ;  but  hère  also  is  an  exceptional  fact 
which  deserves  to  be  pointed  out  :  contrary  to  the  rule,  the  muscles 
of  the  extremities,  and  particularly  those  of  the  thenar  and  hypo- 
thenar  eminences  and  of  the  forearms,  are  comparatively  little 
affected.  Per  contra,  the  lésions  were  relatively  great  in  the  deltoid 
and  the  trapezius,  especially  on  the  left  side,  in  diff'erent  muscles  of 
the  larjnx,  and  lastly,  in  the  tongue.  It  is  important  to  remark 
that  this  mode  of  distribution  could  only  be  revealed  by  a  necro- 
scopic  examination,  for,  during  life,  the  left  shoulder  was  the  only 
part  of  the  body  where  clinical  observation  was  able  to  detect 
partial  atrophy  of  the  muscular  masses  of  some  little  extent.  The 
tongue,  especially,  it  is  explicitly  stated  in  the  observation,  had 
retained  its  thickness,  its  dimensions,  its  smooth  surface,  and,  in  a 
word,  ail  the  appearances  of  the  normal  state,  although  its  proper 
muscles  contained  a  rather  large  number  of  degenerate  or  wasted 
primary  fibres,  and  that  its  movements  were  otherwise  remarkably 
impeded.  On,  the  whole,  putting  aside  the  information  supplied  by 
the  partial  emaciation  of  the  left  shoulder,  the  generalised  progres- 
sive muscular  atrophy  which,  in  our  observation,  was  found  com- 
bined  with  the  symptoms  of  labio-glosso-laryngeal  paralysis,  might 
hâve  passed  completely  unnoticed,  had  not  attention  been  aroused 
by  the  existence  of  intense  fibrillary  movements  spread  over  nearly 
ail  points  of  the  body. 

Confronting  the  muscular  lésions  in  question  with  the  altérations 
which  the  nerve-cells  présent  in  the  différent  régions  of  the  cord 
and  bulbus,  it  will  be  remembered  that,  betweeu  thèse  and  '  those, 
an  exact  corrélation  exists.  In  both,  the  lésions  are  diffuse,  dis- 
seminated  intimately  amongst  them.  Ouglit  we  to  belle ve  that,  in 
this  case,  the  altérations  of  the  muscular  system  hâve  proceèded 
from  the  lésion  of  the  nerve-centres  along  the  bulbar  and  rachidian 
nerves  ?  The  arguments  in  favour  of  this  opinion  hâve  been  detailed 
on  several  occasions  in  this  volume  ;  we  do  not  think  it  necessary 
to  reproduce  them  hère  anew,  hence  we  propose  to  admit,  as  a 


PATHOGENY.  375 

probable  hypothesis,  that  sach,  in  fact,  bas  been  the  pathogenic 
mode  of  thèse  morbid  pbenomena.  But  this  being  conceded^  do 
we  possess  ail  the  éléments  necessary  for  the  construction  of  a 
somewhat  satisfactory  theory  of  the  affection  such  as  it  has  shown 
itself  in  the  case  reported  ?  We  do  not  think  so  ;  besides,  know- 
ing  absolutely  nothing  concerning  the  nature  and  origin  of  the 
lésion  of  the  nerve-cells^  there  are  many  other  desiderata  yet  which 
we  might  mention. 

We  shall  only  notice  one  point  ;  it  is  known  that,  in  our  patient, 
the  différent  movements  of  the  tongue,  and  especially  those  con- 
nected  with  the  articulation  of  words  and  déglutition,  were  consi- 
derably  weakened,  and  that,  at  the  autopsy,  we  found  in  the  muscles 
which  constitute  that  organ  lésions  which  were  evidently  insufficient 
to  account  for  so  marked  a  paralytic  condition.  Whence  should  we 
deem  this  motor  impotence,  independent  of  the  trophic  lésion,  to  be 
derived  ?  We  find  nothing  to  refer  to  save  this  same  lésion  of  the 
nerve-cells,  from  which  we  hâve  already  derived  the  nutritive  altéra- 
tion of  the  muscular  fibres,  and  it  is  difficult  to  comprehend  by 
what  mechanism  this  selfsame  lésion  has  been  able  to  produce 
simultaneously  such  différent  effects.  Let  us  remark,  in  passing, 
that  we  cannot  suppose  the  intervention  hère  of  some  peculiar  influ- 
ence of  the  great  sympathetic,  since  we  hâve  to  explain,  this  time, 
not  the  présence  of  trophic  lésions  of  muscles  which  are  accounted 
for  by  the  altération  of  the  hypoglossus-nucleus,  but  the  existence 
of  a  muscular  paràlysis  independent  of  the  atrophy,  at  least  in  part.i 
This  is  a  serious  difficulty,  which  we  hâve  already  met  with  in  con- 
nection with  progressive  muscular  atrophy  and  infantile  spinal 
paràlysis.^  Manifestly,  in  the  présent  state  of  our  knowledge  a 
definite  judgment  cannot  be  pronounced;  let  us,  therefore,  content 
ourselves,  at  présent,  with  registering  the  positive  data  supplied  by 
anatomical  examination,  and  wait  until  new  facts  arrive  to  throw 
light  on  thèse  obscure  questions. 

It  has  been  several  times  already  proposed  to  connect  with  a 
primary  lésion  of  the  grey  nuclei,  situated  iu  the  bulbus,  the  sym- 
ptomatic  groups  known  under  the  name  of  glosso-labio-laryngeal 

'  The  iutegrity  of  the  great  cervical  sympathetic  has,  besides,  been  explicitly 
mentioned  in  one  report, 

"  In  progressive  muscular  atrophy,  muscular  paràlysis  without  atrophy,  and 
atrophy  without  paràlysis,  are  often  found  intermingled  in  the  same  points  ; 
this  is  a  fact  which  has  been  properly  poiuted  ont  by  MM.  floberts  (*  Reyuolds' 


376  GLOSSO-LARYNGEAL  PARALYSIS. 

paralysis.^  Pathological  anatomy  cornes  to-day;  to  offer  décisive 
support  to  this  hypothesis,  which  hitherto  was  based  on  physio- 
logical  induction.  But,  it  is  not  at  ail  certain  that  ail  the  clinical 
facts  to  which  this  dénomination  may  be  given  are  identical,  and 
acknowledge  the  same  origin.  It  is  easy  to  foresee,  that  coarse 
lésions  of  the  bulbus,  such  as  a  tumour,  a  diffuse  swelling,  might 
under  certain  determined  circumstances  produce  very  nearly  the 
same  effects  as  the  primary  atrophy  of  the  nerve-cells.  On  the 
other  hand,  it  is  évident  that  in  the  cases  in  which  the  altération 
bears,  not  only  on  the  original  nuclei,  but  also  on  the  nerve  trunks 
after  emerging  from  the  bulbus,  should  form  a  category  apart.  In 
truth,  for  want  of  complète  examination  of  the  bulbus,  the  reality 
of  cases  of  this  kind  is  not  yet  sufficiently  established.  Lastly,  the 
absolute  anatomical  integrity  of  the  paralysed  muscles,  several 
times  mentioned  by  skilful  observers  seems,  in  its  turn,  to  suggest 
an  important  distinction.  I  would,  however,  point  out,  in  connec- 
tion with  this,  that  in  such  a  case  the  absence  of  granulo-fatty 
altération  of  the  muscular  fibres  has,  most  frequently,  been  alone 
distinctly  affirmed.  Now,  we  know,  by  rather  numerous  examples, 
that  the  best  characterised  progressive  muscular  atrophy  may  reach 
its  final  term,  without  the  primary  fibres  losing  their  cross  strise  or 
showing  the  least  trace  of  granular  fatty  degeneration.  The  multi- 
plication of  the  sarcolemma-nuclei,  and  the  more  or  less  marked 
réduction  of  the  diameter  of  a  certain  number  of  primary  fibres  are 
then  the  only  muscular  altérations  which  histological  examination 
allows  us  to  remark. 

In  conclusion,  I  would  request  attention  to  the  very  remarkable 
circulation  disturbance  which,  in  our  patientas  case,  marked  the  last 
days  of  her  life.  The  puise  beat  130  to  150  times  a  minute,  with- 
out the  thermometer  marking  the  slightest  increase  of  the  central 
température.  This  disorder  of  the  movements  of  the  heart  was 
accompanied  by  a  quite  peculiar  feeling  of  anxiety,  of  which  the 
Word  dyspnœa  would  give  but  a  very  imperfect  idea.     Thèse  phe- 

System  of  Medicine/  t.  ii,  p.  171,  1867),  Duménil  of  Rouen  ('Atrophy 
musculaire  graisseuse  progressive,'  pp.  93  et  108,  Rouen,  1867),  and  more 
recently  Her»  Benedikt  ('  Elektro  thérapie,'  p.  385,  Wien,  1868).  It  is 
observed  in  the  simplest  cases  of  progressive  amyotrophy,  where  there  exists 
no  sign  of  any  lésion  of  the  white  columus  of  the  spinal  cord. 

^  See,  amongst  others,  A.  Waclismith,  'Ueber  Progressive Bul bar  Paralysie, 
&c.,'  Dorpat,  1854;  aud  '  Centralblatt,'  1864;  L.  Clarke,  'Researches  on 
the  Intimate  Structure  of  the  Brain,'  2ad  séries,  1868,  p.  318. 


DISORDERS  OP   CIRCULATION.  377 

nomena  recall  those  which  liave  frequently  been  remarked  in  man, 
in  cases  in  which  the  action  of  the  pneumogastric  nerves  was 
impeded  in  conséquence  of  the  compression  exercised  by  a  tumour 
of  the  mediastinum  ;  the  altération  of  the  origin-nuclei  of  the  pneu- 
mogastric nerves  which  the  examination  of  the  bulbus  has  revealed 
in  our  case,  appears  to  us  to  account  for  those  cardiac  disorders 
which  hâve  not  a  little  contributed,  undoubtedly,  to  détermine  the 
fatal  termination. 

(Extract  from  the  '  Archives  de  Physiologie  normale  et  patholo- 
gique/ 1870,  p.  247.) 


VI. 

NOTE  UPON  THE  ANATOMICAL  STATE  OE  THE  MUSCLES  AND 
SPINAL  CORD  IN  A  CASE  OF  PSEUDO-HYPERTROPHIC 
PARALYSIS.    By  J.  M.  Charcot. 

(See  Lecture  XIV,  p.  205.) 

SoME  months  ago,  my  friend,  M.  Duchenne  (de  Boulogne),  gave 
me  several  anatomical  fragments,  requesting  me  to  examine  them  ; 
they  were  obtained  from  a  young  patient,  sufPering  from  tlie  affec- 
tion described  under  the  name  of  pseudo-hypertrophie  or  myoscler- 
otîc  paralysis,  and  wlio  had  succumbed,  some  weeks  before,  in  the 
Hôpital  Sainte-Eugénie,  in  the  wards  of  Dr.  Bergeron,  in  consé- 
quence of  an  intercurrent  disease.  The  clinical  history  of  the 
little  patient  in  question  is  well  known  ;  it  has  been  traced  with 
great  care  by  Dr.  Bergeron,  in  a  communication  made  to  the 
Société  Médicale  des  Hôpitaux,  in  1867.^  M.  Duchenne  (de  Bou- 
logne) has  reproduced  it  in  his  memoir  on  pseudo-hypertrophie 
muscular  paralysis.^  A  good  full-length  photograph  of  the  patient, 
annexed  to  the  communication  of  M.  Bergeron,  shows  the  exagge- 
rated  prominence  presented  by  most  of  the  muscular  masses  in  the 
child  in  question,  and  shows  perfectly  the  characteristic  attitude 
wliich  he  assumed,  when  standing  erect.^  For  ail  that  concerns 
the  clinical  history  of  the  case,  I  refer  the  reader  to  the  works  just 
quoted,  and  I  wish  to  confine  myself  to  describing,  in  the  présent 
note,  the  anatomical  facts  which  I  hâve  been  able  to  ascertain,  with 
the  assistance  of  my  house  surgeon,  M.  Pierret.  Some  short  re- 
marks  concerning   the   pathologicai  anatomy   and   physiology   of 

^  '  Bulletins  et  Mémoires  de  la  Société  Médicale  des  Hôpitaux  de  Paris,' 
t.  iv,  ji'c  série,  1867,  p.  157,  Communication  faite  le  24  Mai,  avec  une  photo- 
graphie. 

-  Extract  from  the  '  Archives  Générales  de  Médecine,'  Nos.  de  Janvier, 
1868  et  suivants,  p.  19,  Obs.  xii. 

^  See  also  the  figures  3,  4,  and  9  of  the  memoir  of  M.  Duchenne  (de 
Boulogne). 


LESIONS  OF   MUSCLES.  379 

pseudo -hypertrophie  paralysis  will  follow  the  principal  points  of  the 
statement  and  serve  as  its  corollary, 

The  fragments  which  I  possess  had  ail  been  hardened  in  chromic 
acid;  they  comprise — 1°,  différent  fragments  of  the  deltoid,  psoas, 
pectoral,  and  sacro-lumbar  muscles  ;  2°,  the  cervical  enlargement 
of  the  superior  half  of  the  dorsal  région  of  the  spinal  cord  ;  3°, 
varions  morsels  of  the  sciatic,  médian,  and  radial  nerves  ;  4°,  a 
fragment  of  the  muscular  wall  of  the  left  ventricle. 


In  the  first  place  I  shall  mention  what  concerns  the  external 
muscles.  As  folio ws  from  the  détails  of  the  chnical  report,  the 
pectorals  and  sacro-lumbar  muscles  had,  so  to  speak,  alone  escaped 
the  apparent  hypertrophy  which,  at  a  given  moment,  had  seized 
upon  the  greater  part  of  the  muscular  masses  ;  the  same  may  be 
said  with  respect  to  the  psoas,  which,  on  autopsy,  were  found  to  be 
rather  reduced  in  size.  The  altérations  exhibited  by  thèse  muscles 
may  be  considered  as  representing  the  first  phases  of  the  morbid 
process  ;  the  ultimate  phases,  on  the  contrary,  could  be  studied  in 
the  deltoid  muscles,  which  were  distinguished,  during  life,  by  a  very 
marked  increase  of  volume. 

The  examination  of  the  hardened  fragments,  with  the  naked  eye, 
enables  us  already  to  observe  a  first  distinction  ;  thus,  whilst  the 
fragments  of  the  deltoid  exhibit,  in  the  sections,  a  yellowish  colour, 
having  the  appearance  and  consistence  of  a  lardaceous  mass — phe- 
nomena  plainly  owing  to  the  interposition  of  a  large  quantity  of 
fatty  tissue — the  psoas,  sacro-lumbar,  and  pectoral  muscles,  in  their 
turn,  présent  very  nearly  the  appearance  of  normal  muscles,  treated 
under  the  same  conditions  by  chromic  acid,  but  with  an  evidently 
firmer  consistence,  and  a  résistance  which  recalls  that  of  fibrous 
tissue. 

The  histological  altérations  of  thèse  muscles  consist  in  this  :  on 
transverse  sections,  what  first  strikes  us,  in  the  psoas,  for  instance, 
where  the  lésion  is  least,  is  that  the  thin  laminse  of  connective 
tissue  (appurtenances  of  the  jîmmysizw^ï  internum),  which,  in  the 
normal  state,  barely  séparâtes  the  primary  muscular  fibres  and  leaves 
them  almost  in  contact  with  each  other,  are  hère  replaced  by  thick 
septa,  the  narrow  diameter  of  which  equals,  in  some  parts,  or  even 
exceeds,  that  of  the  muscular  fibres.     Thèse  septa^  as  we  can  make 


380 


PSEUDO-HYPEETEOPHIC    PAEALYSIS. 


sure,  especially,  by  the  examination  of  shredded  longitudinal  sec- 
tions, are  constituted  by  connective  tissue  of  récent  formation,  where 
tlie  laminate  fibres,  directed  chiefly  parallel  to  the  long  axis  of  the 
muscular  fibres,,  are  intermingled  with  embryoplastic  nuclei  and 
fusiform  cells  in  considérable  number.  In  other  muscles,  as  the 
pectoral,  and  the  sacro-lumbar  muscles,  where  the  évolution  of  the 
altération  appears  to  be  more  advanced,  the  nuclei  and  the  cells  hâve 
diminished  in  number  or  seem  to  hâve  disappeared,  and  the  septa 
are  almost  exclusively  formed  by  bundles  of  long  wavy  fibres,  dis- 
posed  parallel  to  each  other,  with  very  distinct  and  well-marked 
outlines. 

The  interposition  of  adipose  cells  between  thèse  fibrillse  marks 
a  new  phase  of  the  process  (fig.  35) .  The  fat  cells  are  sparse,  at 
first,  isolated,  and  lost,  as  it  were,  in  the  midst  of  bundles  of  fibrillse  ; 
but  their  number  augments,  in  certain  points,  in  such  proportion 
that  they  substitute  themselves  for  the  fibrils,  which  in  the  end  com- 
pletely  disappear.  This  fatty  substitution,  sketched  out  already  in 
some  parts  in  the   non-hypertrophied    muscles,    becomes  almost 


I-,  ^ 


-Pïf^'  35- — Transverse  section  of  a  muscle  in  pseudo-hypertrophie  'par aly sis  (intcr- 
mediate  phase  between  the  first  and  second  period  of  tlie  process).  i,  i, 
islets  of  connective  tissue  ;  m,  m,  sections  of  muscular  bundles  ;  G,  G,  adi- 
pose cells. 

gênerai  in  the  deltoid,  where  the  augmentation  of  size  was,  it  is 
known,  well  marked.  In  fact,  the  microscopic  examination  of  this 
muscle  shows  the  greater  part  of  the  surface  of  the  transverse  sec- 
tion to  be  occupied  by  adipose  cells,  almost  contiguous  throughout, 
heaped  against  each  other,  and  made  polyhedric  by  pressure;  hère 
and  there,  in  the  midst  of  the  adipose  tissue,  we  me€t  entire  islets 


LESIONS   OF    MUSCLES. 


381 


composed  of  several  primary  muscular  fibres  (from  t wo  to  eight,  teu, 
or  twelve  at  most),  and  completely  enveloped  with  fibrillae,  or  isolated 
fibrillary  tracts  witliout  muscular  fibres  ;  or,  lastly,  and  most  rareiy, 
isolated  muscular  fibres,  deprived  of  tlieir  fibrillary  envelope,  and 
placed  in  immédiate  relation  with  the  cells  of  the  adipose  tissue 
(fig.  36).  But,  I  repeat,  everywhere,  in  the  deltoid,  is  the  fatty 
tissue  predominating.  The  islets  composed  of  fibrillary  connective 
tissue  and  of  primary  muscular  bundles  are  only  seen  hère  and  there, 
at  long  intervais,  and,  in  certain  parts,  they  are  even  altogether  dé- 
ficient ;  on  the  contrary,  in  the  pectoral  muscles,  and  in  the  sacro- 
lumbar  masses,  the  présence  of  fatty  cells  is  a  rare  and  accidentai 
fact  :  in  the  psoas,  where  the  altération  is  seen  in  its  first  stage, 
there  are  no  traces  of  it. 

In  short,  fatty  substitution  evidently  represents  the  last  phase  of 
the  morbid  process,  and  in  proportion  as  it  advances  the  fibrillary 
tissue  of  new  formation,  as  well  as  the  muscular  bundles,  tends  to  dis- 
appear.  It  is  time  now  to  seek  out  the  mode  by  which  this  disap- 
pearance  of  muscular  fibres  takes  place  ;  it  is  already  noticeable  in 
the  first  period,  when  the  interstitial  connective  tissue  begiiis  to 


Fig.  36. — Longitudinal  section  of  a  muscle  in  pseudo-hypertrophie  paralysis 
(second  period  of  the  morbid  process).  Adipose  cells  every where  in 
contact,  and  rendered  polvhedrical  by  pressure.  Isolated  muscular  fibres, 
deprived  of  their  fibrillary  envelope  and  placed  on  immédiate  relation 
with  tlie  cells  of  the  fatty  tissue.  The  muscular  bundles,  even  the 
slenderest,  hâve  preserved  their  cross  striation. 

show  hype^plasia,  irrespective  of  any  trace  of  fatty  substitution. 
Thus,  in  the  psoas,  on  transverse  sections,  the  muscular  bundles. 


382  PSEUDO-LAETNGEAL  PARALYSIS. 

surrounded  on  ail  sides  by  considerably  thickened  septa  of  perî- 
mysiwni  intermim,  appear  at  first  sight  to  hâve  preserved  nearlv  ail 
tlieir  dimensions  and  other  cliaracters  of  tlie  normal  state  ;  but  a 
less  superficial  examination  soon  shows  that  a  good  number  of  thèse 
bundies  hâve  suffered  a  more  or  less  marked  diminution  in  dia- 
meter  ;  many  even  are  so  much  wasted  that  it  requires  the  closest 
scrutiny  to  discern  them  in  the  substance  of  the  interstitial  con- 
nective  tissue. 

The  examination  of  longitudinal  sections,  and  especially  of  those 
prepared  by  dilaceration,  complètes  thèse  data  :  the  greater  part  of 
the  muscular  bundies,  even  those  which  hâve  undergone  very  well- 
marked-atrophy,  préserve  to  the  last  limits  of  emaciation  most  dis- 
tinct transverse  striation.  Neither  the  sarcolemna-sheath,  nor  the 
nuclei  which  it  encloses,  présent  any  altération,  and,  as  to  the 
muscular  substance,  no  trace  of  granulo-fatty  degeneration  is  there 
observed.  Such  is  the  rule  ;  hère  and  there,  however,  we  meet 
some  bundies,  few  indeed  in  number,  where  the  cross  striœ  are 
absent,  wlrilst  a  longitudinal  striation  has  become  very  apparent  ; 
other  bundies,  absolutely  devoid  of  ail  striation,  whether  transverse 
or  longitudinal,  hâve  a  hyaline  appearance  and  are  charged  with 
granulations  ;  lastly,  there  are  others, — which  are  always  of  the 
smallest  diameter, — the  muscular  substance  of  which  seems  to  be 
broken  into  fragments  in  which  the  transverse  striation  is  still  very 
manifest,  and  in  the  interspaces  of  which  more  or  less  numerous 
clusters  of  nuclei  are  accumulated,  which  distend  the  sheath  of  the 
sarcolemma.  But,  in  short,  it  is  rare  that  muscular  bundies,  on 
the  way  to  destruction,  exhibit  any  one  of  thèse  modes  of  altération. 
The  greater  part  of  them  do  only  présent,  to  the  final  term,  the 
characters  of  simple  atrophy,  without  multiplication  of  nuclei  and 
with  persistence  of  transverse  striation. 

In  the  deltoid  the  muscular  bundies  are  found  with  the  same 
appearances  ;  only  those  of  them  which  hâve  preserved  their  normal 
diameter  are  much  more  rare.  Most  of  thèse  hâve  suffered  mani- 
fest atrophy;  many  of  them  are  remarkable  for  their  extrême 
slenderness.^  The  hyaline  state,  with  fatty  granular  degeneration, 
segmentation  of  the  muscular  substance  with  multiplication  of  the 

^  Measurements  give  : — 1°,  for  the  primary  fibres  in  tlie  psoas  muscle, 
transverse  diameter,  o'0429™"'",  o"026"'"'",  o'oo66"""-,  o'0O33""""  (fibres  ex- 
hibiting  the  last  dimensions  are  rare)  ;  2°,  in  the  deltoid,  o'o/""'*,  o'oi2'"'"', 
oo'o66™'°',  o"0933"""*,  ^^^  under. 


LESIONS   OF   MUSCLES.  383 

nuclei  of  the  sarcolemma  are  hère,  perhaps,  more  fréquent  than 
elsewhere,  but  it  is  still  simple  atrophy  which  is  always  suprême. 
With  respect  to  the  pectoral  and  sacro-lumbar  muscles,  the  lésions 
of  the  primary  fibres  which  are  there  met  with  hold  a  médium 
between  the  two  extrêmes,  and  allow  the  transition  to  be  traced  out. 

Keeping  in  mind  the  results  just  stated,  one  might  endeavour,  we 
believe,  to  reconstruct,  at  least  in  a  most  gênerai  way,  the  mode  of 
évolution  of  muscular  altération  proper  to  pseudo-hypertrophie 
paralysis.  At  the  commencement,  besides  the  thickening  of  the 
walls  of  the  vessels,  connective  hyperplasia  and  simple  atrophy  of  a 
certain  number  of  muscular  fibres  are  the  only  lésions  observed. 
At  this  period,  fatty  substitution  is  either  totally  absent,  or  else 
it  plays  an  evidently  accessory  part.  This  first  phase  seems  to 
correspond  with  the  first  clinical  stage,  noticed  by  ail  observers — 
a  period  in  which  the  only  appréciable  symptoms  consist  in  the 
more  or  less  marked  weakness  of  certain  muscles,  which  do  not  as 
yet  exhibit  any  apparent  hypertrophy,^  or  which  even  sometimes 
show  themselves  manifestly  atrophied.- 

What  takes  place  in  the  second  period  of  the  disease,  when  the 
paralysed  muscles  begin  to  increase  in  volume?  According  to 
M.  Duchenne  (de  Boulogne)  the  apparent  hypertrophy  in  ques- 
tion would  be  the  conséquence  of  connective  hyperplasia.  "  It  is 
this,"  he  says,  "  which  produces  the  augmentation  in  size  of  the 
muscles  in  direct  ratio  to  the  quantity  of  connective  and  fibroid 
interstitial  tissue  produced  in  hyperplasia."  This  opinion  is  based 
on  the  results  repeatedly  obtained  from  examination  of  morsels  of 
muscle  extracted,  during  life,  by  means  of  the  emporte-pièce  hisio- 
logique;^  but  it  may  be  questioned  whether,  in  this  little  opération 
the  islets  of  connective  tissue  are  not  withdrawn  in  préférence  bv 
the  instrument,  which,  on  the  contrary,  would  find  much  more 
difficulty  in  laying  hold  of  the  clusters  of  adipose  cells.  It  is 
certain  that  in  cases  where  fragments  of  muscles  hâve  been  removed 
during  life  by  excision,  they  hâve  always  exhibited,  to  a  high  degree, 
the  characters  of  fatty  substitution.* 

1  Duchenne  (de  Boulogne),  '  Electrisation  localisée,'  3^  édit.,  p.  605. 

^  Pepper,  '  Clinical  Lecture  on  a  Case  of  Progressive  Muscular  Sclerosis,' 
Philadelphia,  1871,  pp.  14  and  16. 

3  Duchenne  (de  Boulogne),  loc.  cit.,  p.  603  ;  Foster,  '  The  Lancet,'  May 
8,  1869,  p.  630. 

''  Griesinger  and  Billroth,  Heller  and  Zenker,  Wernich,  vide  Siedel,  '  Die 
Atrophia  Musculorum  Lipomatosa,'  Jcna,  1867. 


384  PSEUDO-LARYNGEAL  PAEALYSIS. 

The  impression  wiiicli  remains  on  my  mind,  after  often-repeated 
examinations  of  the  fragments  confided  to  me,  is  tliat  hyperplasia  of 
the  connective  tissue  and  atropliy  of  the  muscular  fibres  advance,  as 
it  were,  with  equal  steps,  the  latter  showing  itself  ail  the  more 
gênerai  and  marked  in  proportion  as  the  former  is  itself  more  deve- 
loped  ;  so  that  the  production  of  connective  tissue  would  be  in  some 
sort  proportionate  to  the  extent  of  void  left  by  the  atrophy  or  dis- 
appearance  of  the  muscular  fibres.  It  is,  however,  possible  that 
the  connective  hyperplasia  may  sometimes  assume  the  upper  hand, 
and  thus  produce  a  certain  degree  of  apparent  hypertrophy  ;  but  I 
find  it  difficult  to  understand  how  it  could  ever  explain  the  often 
enormous  increase  in  size  presented  by  the  muscular  masses  at  a 
certain  epoch  of  the  disease,  and  I  am  led  to  believe  that  substitu- 
tion of  adipose  tissue  hère  plays  the  prédominant  part.  However 
this  be,  I  am  ready  to  acknovrledge  that  the  question  which  I  hâve 
just  raised  cannot  yet  receive  a  definite  solution. 

In  what  does  the  morbid  process  consist  which,  in  pseudo-hyper- 
trophie paralysis,  détermines  the  altération  of  the  muscular  tissue  ? 
I  am  struck,  like  many  others,  with  the  analogies  which  exist 
betvveen  this  altération  and  that  which,  where  the  viscera  are  con- 
cerned,  is  generally  designated  under  the  name  of  cirrhosis,  or  again, 
of  sclerosis,  and  I  do  not  see  that  any  serions  objections  hâve  ever 
been  formulated  against  this  approximation.  The  circumstance, 
alone,  that  invasion  of  fatty  tissue  takes  place,  at  a  certain  epoch  of 
the  disease,  in  a  fated  manner,  at  least  in  some  muscles,  appears  to 
me  to  constitute,  in  the  case  in  point,  a  really  distinctive  character, 
so  that  the  dénomination  of  myosclerotic  paralysis,  proposed  by 
Duchenne  (de  Boulogne),  can  only  strictly  be  apphed  to  the  first 
periods  of  the  disease,  whilst  those  of  atrophia  musculorum  lipoma- 
tosa  (Seidel),  and  of  Upomatosis  Uixurians  (Heller),  generally  used 
by  German  authors,  are  appropriate  only  to  advanced  periods.  But 
I  do  not  wish  to  insist  further  on  this  point  ;  at  présent  I  shall 
confine  myself  to  the  examination  which  I  hâve  made  of  the  spinal 
cord. 


IL 

The  récent  researches  relating  to  the  pathological  anatomy  and 
physiology  of  spontaneous  amyotrophies  bave  allowed  us,  as  is 
known,  to  connect  with  a  lésion  of  certain  well-determined  régions 


STATE    OF    SPINAL    CORD.  iiS5 

of  the  spinal  cord,  a  considérable  number  of  tliese  affections. 
Latterly  the  opinion  bas  been  several  times  put  forth  that  pseudo- 
liypertrophic  paralysis  M'hich,  in  some  respects,  approximates  to 
progressive  muscular  atrophies,  is  also  referable  to  a  spinal  source. 
This  hypothesis  rests  upou  no  solid  foundation,  and  there  even 
exists,  in  scientific  records,  an  observation,  followed  by  necroscopy, 
which  tends  to  completely  invalidate  it.  I  hère  allude  to  the  case 
presented  by  H.  Eulenburg  to  the  Médical  Society  of  Berlin,  in 
which  the  autopsy  was  conducted  by  Dr.  Cohuheim.i  It  is  true 
that,  in  this  case,  the  spinal  cord  having  been  examined  in  the 
fresh  state,  or  after  imperfect  hardening,  very  délicate  lésions — such 
as  atrophy  of  the  motor  nerve-cells  and  sclerosis  of  the  anterior 
cornua  of  grey  substance — might  in  strictness  hâve  escaped  obser- 
vation. In  this  respect,  our  case,  on  the  contrary,  leaves  nothing 
to  be  desired,  and  it  pleads  absolutely  in  the  same  direction  as  that 
of  Dr.  Cohnheim. 

Although  we  hâve  only  liad  in  our  hands,  a  portion  of  the  cord 
comprising  the  upper  half  of  the  dorsal  région,  and  the  entire 
cervical  enlargement,  the  results  which  we  obtained  from  our 
examination  are  not  the  less  very  significant.  It  must  not,  in  fact, 
be  forgotten  that  the  muscles  which  receive  their  nerves  from  the 
last-named  région  of  the  cord  were  mostly  affected  to  a  high  degree, 
and  that  the  deltoid  muscles,  amongst  others,  exhibited  in  the  most 
distinct  manner  the  characters  of  hypertrophy  from  fatty  substitu- 
tion. If,  therefore,  in  this  case,  the  muscular  lésions  had  been 
connected  with  spinal  lésions,  the  latter  would  not  hâve  failed  to 
show  themselves  well  marked  in  the  cervical  enlargement  of  the 
spinal  cord. 

Our  observations  hâve  been  made  on  transverse  sections  coloured 
with  carminé,  and  figured  with  great  skill  by  M.  Pierret.  Thèse 
sections,  also,  hâve  been  very  numerous  and  were  taken  from  the 
most  varions  parts  of  the  cervical  and  dorsal  régions.  Now,  the 
resuit  lias  been  absolutely  négative  ;  throughout  we  hâve  found  the 
antero-lateral  and  posterior  whlte  columns  in  a  state  of  perfect 
integrity  ;  the  grey  substance  which  we  hâve  made  the  spécial  object 
of  our  investigations  did  not  exhibit  any  trace  of  altération.  The 
anterior  cornua  were  ueither  atrophied  nor  deformed  ;  their  neuroglia 
had  retained  its  usual   transparency,  and  the  motor  nerve-cells, 

^  '  Verhandlungeu  der  Berliuer  Mediciuiscliea  Gesellschaft,' Berlin,  1866 
Heft.  2,  p.  191. 

VOL.  II.  25 


386  PSEUDO-HYPEETEOPHIC    PAEALYSIS. 

normal  in  number,  did  not  exhibit,  in  their  varions  constitnent 
parts,  any  déviation  from  the  physiological  type.  Let  us,  îastly, 
add  that  the  spinal  roots,  botli  anterior  and  posterior,  appeared 
also  perfectly  liealthy. 

I  do  not  think  it  necessary  to  insist  furtlier  to  show  the  interest 
which,  as  regards  the  question  that  occupies  us,  belongs  to  thèse 
necroscopic  facts,  corroborated  as  they  are  by  the  previous 
observation  of  MM.  Eulenburg  and  Cohnheim;  if  I  do  not 
deceive  myself,  the  conclusions  to  which  they  naturally  lead  is, 
that,  according  to  ail  probability,  pseudo-liypertropUc  paralysis 
ougJd  to  he  coiisidered  as  independent  of  ail  appréciable  lésion  of  the 
spinal  cord  or  nerve-roots. 

An  observation  recently  published  in  the  ^Archiv  der  Heil- 
kunde,'^  by  H.  O.  Barth,  assistant  at  the  Pathological  Institute  of 
Leipzig,  seems  to  be  in  formai  contradiction  with  the  proposition 
that  has  just  been  formulated.  This  case  is,  indeed,  referred  by 
the  author  to  pseudo-hypertrophie  paralysis,  and  it  is  followed  by 
an  account  of  the  autopsy,  made  with  the  greatest  care,  where  the 
existence  of  very  marked  spinal  lésions  is  placed  beyond  doubt  ;  but 
Ido  not  believe  that  this  case  has  the  signification  givento  it, — far 
from  it.  The  case  is  that  of  a  mau,  aged  about  forty-four,  who  ex- 
perienced  in  1867,  three  years  before  the  fatal  termination,  the  first 
symptoms  of  motor  paralysis  in  the  lower  extremities.  The  para- 
lysis  grew  prpgressively  worse  and  spread  to  the  upper  extremities. 
Two  years  after  its  beginning,  the  patient  was  condemned  to 
remain  in  bed,  and  was  deprived  of  power  of  most  movements. 
As  the  paralysis  of  motion  advanced,  more  or  less  sharp  pains  and 
disagreeable  formications  occupied  the  limbs  ;  moreover,  the  para- 
lysed  muscles  showed  great  atrophy  and,  in  some  points,  became  the 
seat  of  very  well-marked  fibrillary  contractions.  Pinally,  vocal  and 
deglutition-movements  grew  difficult.  During  the  course  of  the  last 
months  of  his  life,  many  of  the  atrophied  muscles,  the  adductors  of 
the  thumb  and  the  muscles  of  the  calf  in  particular,  underwent 
remarkable  increase  in  size,  although  motor  impotence  persisted  to 
the  same  degree  as  before.  At  the  autopsy,  the  muscles  of  the 
members  generally  cxhibited,  in  varying  degrees,  the  characters  of 
fatty  substitution.  Some  of  the  muscle-fibres  presented  the  altéra- 
tions of  simple  atrophy,  others  and  fewer  showed  those  of  granulo- 

'  Otto  Bartb,  "  Beitiâgc  zur  Kenntniss  der  Atrophia  Musculorum  Lipoma- 
tosa,'  iu  '  Arcliiv  der  Heilkunde,'  Leipzig,  1871,  p.  120. 


STATE    OP    SPINAL    CORD.  387- 

fatty  degeneratioii.  There  also  exists,  in  several  points,  in  the 
intervais  of  thèse  fibres,  a  certain  degree  of  connective  hjperplasia. 

An  examination  of  the  spinal  cord  yielded  interesting  results  : 
the  latéral  columns  were  sclerosed,  symmetricallj,  throughout  their 
entire  height,  from  the  upper  end  of  the  cervical  enlargement  to  the 
lower  extremity  of  the  lumbar  région  ;  the  anterior  cornua  of  the 
grey  substance  were  manifestly  atrophied;  in  addition,  a  good  number 
of  the  great  motor  nerve-cells  exhibited  more  or  less  marked  wast- 
ing,  and  many  of  them  had  even  disappeared.  Lastly,  it  was 
remarked  that  a  large  quantity  of  adipose  tissue  had  been  accumu- 
lated  under  the  skin  of  the  limbs,  and  on  the  surface  of  most  of  the 
viscera. 

It  seems  to  me  quite  illegitimate  to  refer  the  case,  of  which  I 
bave  just  sketched,  in  a  very  brief  way,  the  principal  traits,  to  the 
classic  type  of  pseudo-hypertrophie  paralysis.  The  relatively  ad- 
vanced  âge  of  the  patient,  the  existence  of  sharp  pains,  and  formica- 
tion  in  the  limbs,  the  fibrillary  contracture,  difficulty  of  articulation 
and  swallowing  supervening  at  a  certain  stage  of  the  disease,  ail 
thèse  circumstances,  amongst  others,  protest,  if  need  be,  against  such 
an  assimilation.  They  very  naturally  connect  themselves,  on  the 
contrary,  witli  the  morbid  type  to  which  I  hâve  called  •  attention  in 
my  lectures,  in  which  (as  took  place  in  M.  Barth's  case)  sym- 
metrical  sclerosis  of  the  latéral  columns  was  combined  with  pro- 
gressive atrophy  of  the  nerve-cells  of  the  anterior  cornuaJ  Un- 
questionably,  the  muscular  lésions  described  in  the  case  of  Dr.  O. 
Barth's  recall  in  many  respects  those  which  are  found  uniformly 
noticed  in  ail  cases  of  pseudo-hypertrophie  paralysis  hitherto 
published  ;  but  this  circumstance  would  not,  of  itself  alone,  justify  a 
nosographic  approximation.  I  think  I  should  in  référence  to  this 
substance  make  a  remark  which  might  appear  commonplace,  if  the 
fact  to  which  it  is  applicable  did  not  seem  to  hâve  been  sometimes 
misunderstood  :  it  is  that  none  of  the  muscular  lésions  in  question 
belong  peculiarly  to  pseudo-hypertrophie  paralysis,  and  could  not, 
consequently,  suffice  to  differentiate  it.  Thus,  hypertrophy  of  the 
interstitial  connective  tissue  with  simple  atrophy  of  the  muscular 
fibres  is  found,  for  instance,^  after  traumatic  nerve-lesions,  and  in 

1  "  Deux  cas  d'atrophie  musculaire  proi^ressive,  avec  lésion  de  la  sub- 
stance grise  et  des  faisceaux  antéro-latéraux  de  la  moelle  épinière,"  par  MM. 
Charcot  et  A.  Joffroy  ('Archives  de  Physiolog-ie,'  1869,  t.  ii,  p.  334). 

2  Mantegazza,  '  Gazetta  Lomb./  p.  18,  1867;  Erb,  "  Zur  Physiologie  und 


388  PSEUPO-HYPEETROPHIC     PARALYSIS. 

some  cases  of  spinal  infantile  paralysis  ;^  as  to  fatty  substitution,  with 
or  witliout  increase  in  size  of  the  nuclei,  it  may  take  place  as  an  even- 
tual  complication  in  infantile  paralysis  also,3  in  progressive  mus- 
cular  atrophy/  in  spinal  paralysis  of  tlie  adult,^  and  in  many  other 
circumstances  whicli  would  take  too  long  to  enumerate  hère.  It  is 
to  be  remarked  that,  in  sucli  cases,  the  fatty  substitution  of  the 
muscles  appears  to  be  sometimes  connected  with  a  generalised 
lipomatosis,  wliich  shows  itself  particularly,  as  exemplified  by  H. 
Barth's  case,  by  an  accumulation  of  adipose  tissue  under  the  skin 
and  in  the  viscéral  cavities.  Quite  recently,  Dr.  W.  Miiller  has- 
justly  insisted  on  this  point,  in  an  interesting  collection  of  observa- 
tions relating  to  the  pathological  anatomy  and  physiology  of  the 
spinal  cord.'^  But,  I  must  separate  completely  from  the  autîhor 
m-entioned  when,  refusing  ail  autonomy  to  pseudo-hypertrophie 
paralysis,  he  maintains  that  ail  the  cases  that  hâve  been  (arti- 
ficially,  according  to  hira)  grouped  under  this  name  might  critically 
be  referred  to  some  one  of  the  forms  of  amyotrophy  due  to  atrophy 
of  the  motor  nerve-cells.  Nothing,  in  my  judgment,  is  less  justifi- 
able than  this  opinion,  and  the  very  case  which  forms  the  object  of 
the  présent  note  would,  of  itself,  be  sufficient  to  show  its  inauity. 

After  recognising  that  the  muscular  altérations  in  pseudo-hyper- 
trophie paralysis  do  not  dépend  on  atrophy  of  the  nerve-cells  of  the 
anterior  cornua,  we  hâve  reason  to  inquire  whether  they  should  not 
be  correlated  to  some  lésion  of  the  great  sympathetic,  or  the  peri- 
pheral  nerves.  With  référence  to  the  first  point,  I  can  give  no 
information,  the  great  sympathetic  not  having  been  amongst  the 
spécimens  which  were  at  my  disposai.  As  regards  the  second 
point,  I  must  déclare,  after  having  carefally  examined  the  varions 
fragments,  taken  from  the  sciatic,  médian,  and  radial  nerves,  that 
Pathologie,  Anatomie  peripherischer  Paralysen,"  ia  '  Deutsch  Archiv,'  t.  iv, 
1868. 

'  Volkmann,  "Ueber  Kinderlabmung,"  in  '  Sammlung  Kliniscber  Vor- 
tràge,'  Leipzig,  1870;  Charcot  et  Joffroy,  'Archives  de  Physiologie,'  t,  iii, 
1870,  p.  34. 

'  Laborde,  'De  la  Paralysie  de  l'Enfance,'  Paris,  1864;  Prévost, 
'Comptes-rendus  et  Mémoires  de  la  Société  de  Biologie,'  1865,  t.  xvii,  p.  215, 
Paris,  1866;  Charcot  et  Joffroy,  loc.  cit.  ;  Vulpian,  '  Archiv.  de  Physiologie,': 
t.  iii,  1870,  p.  316;  W.  Millier,  'Beitrâge  zur  pathologischen  Anatomie  et 
Physiologie  des  Menschlichen  Riickenmarks,'  No.  2,  Ein  Eall  von  Umschriebe- 
ner  Muskelatropie  mit  Interstitieller  Lipomatose,  Leipzig,  1870. 

^  (and)  ■*  Duchenne  (de  Boulogne),  Communicated  cases. 

5  W.  Miiller,  loc.  cit. 


PERIFHERAL    .NERVES.  389 

thèse  uerves  hâve  appeared  to  me  to  présent,  in  every  part,  the 
appearances  of  the  normal  state.  We  hâve  even  met,  in  the  sub- 
stance of  the  affectée!  muscles,  with  several  nerve-filaments,  which 
seemed  fco  us  exempt  from  altération,  with  the  exception,  however, 
that  in  one  case,  one  of  thèse  filaments  belonging  to  the  psoas 
muscle  displayed,  in  a  thin  carmine-coloured  section,  a  remarkable 
lésion  consisting  of  a  very  marked  hypertrophy  of  the  axis-cylin- 
ders.  On  the  whole,  we  believe  that  before  coming  to  any  décision 
as  to  the  state  of  the  periphieral  nerves  in  pseudo-hypertrophie 
paralysis  it  is  necessary  to  undertake  new  investigations. 

In  terminating,  I  would  point  out,  as  a  fact  worthy  of  interest, 
Ihat  the  muscular  wall  of  the  left  ventricle  of  the  heart  did  not,  in 
our  case,  at  ail  participate  in  the  altérations  which  were  so 
marked  in  the  muscles  of  the  extremities. 

Extract  from  the  '  Archives  de  physiologie  normale  et  patholo- 
gique,' 1871 — 1872,  p.  328. 


YII. 

ON  ATHETOSIS. 

In  one  of  his  last  lectures  at  tlie  Salpêtrière,^  M.  Charcot  bas 
described  the  characters  which  distinguish  a  variety  of  pod-liemi- 
jalegic  hemickorea,  to  whicli  Mr.  W.  Hammond,  of  New  York,  bas 
given  the  name  of  Atlietosis.  But  whilst  Mr.  Hammond,  who  first 
pointed  out  thèse  movements,  considers  them  iii  some  sort  as 
constituting  a  particular  morbid  state,  quite  autonomous,  M.  Charcot 
considers  that  tbey  are  simply  choreiform  movements,  and  that 
tbey  should  be  connected,  nosograpbically,  with  the  bistory  of 
symptomatic  chorea,  as  a  simple  variety.^ 

According  to  Dr.  Hammond,  athetosis"^  is  characterised  by  its 
being  impossible  for  the  patients  to  keep  their  Angers  and  toes  in 
the  position  in  which  tbey  may  be  placed,  and  by  their  continuai 
movement. 

This  définition  is  imperfect,  for  the  following  reasons  : — 1°.  It 
should  be  added  that  the  movements  of  the  fingers  are  performed 
slowly,  and  that  the  fingers  bave  a  tendency  to  assume  constrained 
attitudes  ;  2°,  Moreover,  the  atlietosis  does  not  always  remaiu 
limited  to  the  muscles  which  move  the  fingers  and  toes  ;  sometimes, 
in  fact,  the  entire  hand  and  foot  are  affected.  3°.  Lastly,  in  the 
case  of  one  of  the  patients  whom  Professer  Charcot  bas  shown  to 
his  audience,  some  muscles  of  the  face  and  neck  are  stirred  by 
choreiform  movements,  simultaneously  with  those  of  the  hand  and 
foot. 

The  following  cases  illustrate  perfectly  the  principal  characters 
of  athetosis  : 

Case    j. — Gr — ,   now  aged   32,   had   convulsions   when    eight 

'  December,  1876. 

^  This  opinion  bas  already  been  expressed,  in  au  interesting  work,  by  H. 
Bernliardt,  '  Ueber  den  von  Hammoud  Athelose  gcnanute  Symptomencomplex.' 
3  'AOeroç,  "  without  a  fixed  position." 


ATHETOSIS.  39] 

months  old,  whicli  were  followed  by  paralysis  of  the  left  side. 
From  that  time  until  she  was  six  years  of  âge  slie  had  epileptic  fits 
every  second  month.  They  disappeared  between  the  âge  of  six  and 
nine  ;  tlien  they  came  on  anew,  and  hâve  persisted  ever  since.i 

At  présent  she  is  affected  by  left  hemiplegia,  without  ansesthesia 
or  contracture,  but  with  choreiform  movements  confined  to  the  left 
side  of  the  body^  and  affectiug  the  face,  neck,  hand,  and  foot. 

The  fingers  are  constantly  in  motion;  they  extend  and  flex  in 
succession  and  independently  ;  at  other  times  they  separate  or 
approach,  whilst,  at  the  same  period,  the  wrist  exécutes  various 
movements  of  extension,  pronation,  adduction,  and  abduction. 
Gr —  cannot  keep  lier  fist  closed  ;  immediately  the  fingers  extend 
and  move  in  ail  directions,  the  thumb  is  often  caught  between  the 
two  first  fingers.  She  scarcely  makes  use  of  her  hand  ;  when  she 
has  caught  an  object  she  soon  drops  it  in  conséquence  of  her  fingers 
opening.  When  the  patient  is  standing,  the  foot  at  first  is  at  rest 
upon  the  ground  ;  but,  from  time  to  time,  the  great  toe  séparâtes, 
the  other  toes  rise,  are  flexed,  or  the  heel  is  raised.  Thèse  move- 
ments take  place  every  three  or  four  minutes. 

Let  us  remark  that  there  is  a  species  of  synergy  between  the 
movements  of  the  hand  and  those  of  the  foot  ;  when  the  patient  is 
told  to  open  the  left  hand  or  to  close  it,  the  foot  is  set  in  movement 
each  time,  and  the  toes  are  flexed  or  extended. 

Some  slight  grimaces  are  noticed  in  the  left  half  of  Ûxeface  ;  the 
muscles  of  the  commissures  seem  especially  to  act.  In  the  neck^ 
the  motor  disorder  seems  to  occupy  the  left  platysma  myoides  and 
sterno-cleido-mastoideus. 

Case  2. — Maur — ,  aged  '^'^.  At  nine  months  convulsions,  fol- 
lowed by  paralysis  of  the  left  side.     Epileptic  fits  at  fourteen, 

To-day,  M — ,  in  addition  to  her  epileptic  fits,  exhibits  left  hemi- 
jilegïa,  with  analgesia,  involving  the  face,  trunk,  and  members^  and 
choreiform  movements  occupying  only  the  hand  and  foot  of  the 
paralysed  side  {post-liemiplegic  hemichorea,  variety  athetosis). 

The  joints  of  the  left  upper  extremity  are  stifiF.  The  hand  is 
flexed  upon  the  forearm.  The  fingers  are  unstable,  sometimes  flex- 
ing,  sometimes  extending.  The  habituai  attitude  of  the  two  first 
fingers  seems  to  be  of  forced  extension.   When  the  patient  is  desired 

^  Tor  futher  détails,  see  Raymond,  '  Etude  anatomique,  physiologique,  et 
clinique  de  rHémichorée,'  &c.,  p.  69. 


392  POST-HEMIPLEGIO    HEMICHOEEA. 

to  open  lier  hand,  tlie  fingers  pass  into  forced  extension^  the  tips 
are  turned  back,  and  alinost  immediately  the  fingers  and  the  hand 
are  flexed.  If  she  tries  to  flex  the  thumb  she  can  succeed,  but 
simultaneously,  and  in  spite  of  herself,  the  fingers  are  extended. 
The  large  raovemeuts  of  the  arm  are  not  jerky. 

Foot  tends  to  adduction.  The  great  toe  rises  and  flexes  con- 
tiiiuously.  It  is  the  same  with  respect  to  the  other  toes,  and  their 
movements  are  independent. 

If  the  patient  be  ordered  to  close  or  to  opeu  her  hand,  the  foot  is 
at  once  taken  with  movements  ;  the  heel  rises,  the  toes  stir.  When 
observed  in  bed,  the  patient's  left  hand  and  foot  are  seen  to  be 
nearly  constantly  stirred  by  jerking,  synergie  movements  ;  in  order 
to  lessen  them,  she  holds  the  left  hand  with  the  right. 

Many  times,  during  lectures  previous  to  those  which  we  summa- 
rise,  hère  Professor  Charcot  has  shown  the  patients,  whose  history 
we  hâve  briefly  related  ;  and,  on  his  side,  M.  Raymond  has  published, 
m  extenso,  in  his  thesis,  an  account  of  the  first-mentioned  case. 
Without  a  doubt  thèse  cases,  described  as  belonging  to  hemichorea, 
are  included  in  the  description  of  athetosis,  as  Dr.  Hammond  has 
traced  it.  In  fact,  in  the  second  case,  we  fînd  mention  made  not 
only  of  instability  of  the  fingers,  but  also  of  hemiansesthesia,  under 
an  attenuated  form,  it  is  true,  siuce  there  was  no  analgesia.  This 
coincidence  of  ansesthesia  with  athetosis,  which  has  been  noticed  by 
Dr.  Hammond,  is  a  circumstance  which  might  hâve  been  expected. 
The  opinion  expressed  by  Professor  Charcot  that  we  hâve  hère  a 
simple  variety  oï post- hémiplégie  hemichorea  is,  as  may  be  remarked, 
justified  by  clinical  facts.  Between  our  two  patients  there  is  but  a 
différence  relating  to  the  extent  of  the  abnormal  movements,  limited 
to  the  hand  and  foot  of  the  paralysed  side  in  the  case  of  the  first 
patient  ;  in  that  of  the  second  they,  in  addition,  occupy  the  eor- 
responding  side  of  the  face  and  neck. 

Between  the  latter  patient  and  another  attacked  with  post-hemi- 
plegique  hemichorea,  who  is  also  in  Professor  Charcot's  wards,  the 
différence  again  only  relates  to  the  exte})t,  and  also  to  rhythm  of 
the  involuntary  movements.  The  few  détails  which  we  are  about 
to  give  will  makc  this  rlear  : 

Case  3. — P — ,  at  présent  aged  19,  was  seized,  when  five  years 
old,  with  convulsions  which  lasted  four  hours,  and  werè  foUowed. 


ATHETOSIS.  393 

by  incomplète  paralysis  of  the  left  side.  The  paralysis  is  stated  to 
hâve  disappeared  two  moiiihs  later.  At  the  âge  of  six,  convul- 
sions lasting  five  hours,  iDcomplcte  paralysis  of  the  inembers  of 
the  left  side.  Wheii  seven  and  a  haK  years  old,  convulsions  for 
six  hours,  complète  ^Mrahj sis.  Since  the  time  when  P —  began  to 
use  her  arin  choreifonn  movements  hâve  been  observed.  ïhree 
months  later,  appearance  of  fits  of  partial  epilepsy. 

Présent  state. — Hemiplegia  without  contracture,  but  with  hemi- 
diorea  and  dinainutiou  of  sensibility  on  the  left  side. 

When  the  left  hand  is  closed,  it  is  observed  that  the  Angers 
constantly  want  to  open  out,  and  that  movements  of  supination  and 
pronation  are  superadded  to  thèse  movements.  If  the  hand  be 
open,  movements  of  extension  of  the  fingers  are  observed.  In 
order  to  obtain  a  little  rest,  the  patient  is  obliged  to  press  her 
hand  strongly  against  some  resisting  substance. 

If  the  voluntary  movements  be  considered,  they  are  seen  to  be 
disorderly,  jerking,  abrupt.  Does  the  patient  raise  her  hand  to  her 
face  ?  She  smites  it.  Made  to  take  a  light  object,  she  exécutes  a 
disproportionate  movement  with  the  hand,  and,  at  every  moment, 
threatens  to  let  the  object  fall.  Between  thèse  movements  and 
those  of  chrome  chorea,  the  only  dissimilarity  consists  in  the  fact 
that,  in  the  latter,  the  movements  are  more  rounded,  more  fes- 
tooned,  as  it  were. 

If,  with  Professer  Charcot,  we  confront  thèse  two  patients,  P — 
and  Gr —  (Cases  3  and  i),  we  remark  at  once  that  the  chorei 
form  movements  differ  only  in  so  far  as  regards  their  intensity, 
their  extent,  and  their  rhythm.  In  Case  i  they  are  limited  to  the 
left  half  of  the  face  and  of  the  neck,  to  the  hand  and  foot  of  the 
same  side;  whilst,  in  Case  3,  besides  being  more  abrupt  and 
jerking,  they  affect  ail  the  movements  of  the  members  of  the  left 
side. 

From  the  foregoing  considérations,  it  follows  that  thèse  three 
patients  présent  abnormal  motor  disorders  which  are  similar,  or 
differ  only  in  so  far  as  they  are  more  or  less  extensive,  more  or 
less  rapid,  or  affect  a  less  or  greater  number  of  muscles,  according 
to  the  patient  examined. 

Other  rcasous  there  are  which  corroborate  this  assimilation.  In 
the  three  patients,  the  lésion  is  of  the  same  nature  ;  ail  three  are 
suffering  from  unilatéral  cerehral  atrojihij,  consécutive  on  a  serious 


394  ATHETOSIS. 

encephalic  lésion^  dating  from  childhood  ;  ail  three  are  hémiplégie, 
and  subject  to  fits  of  partial  epilepsy.  Thus,  we  hâve  an  analogy 
in  the  form  of  the  motor  disorders,  and  an  analogy  in  the  conditions 
of  their  development  ;  this  appears  sufficient  to  show  that  athetosis, 
is  only  a  variety  oi post-hemij)legic  hemichorea. 


INDEX. 


Aboetive  cases  of  locomotor  ataxia,  23 

ACHEOMATOPSIA,  40 

Action,  distant,  20 

AcuiTY,  Visual,  40 

Alcoholism,  chronic  (liglitning  pains 

in),  28 
Amaîteosis,  tabetic,  34,  36,  41 
Amblyopia  in  ataxia,  23,  32,  44 
Amyoteophibs,  spinal  ;  varieties,    128 

—  clironic,  163 

—  varieties,  164 

—  protopathic,  164 

—  deuteropathic,  165,  180  (see  ScLE- 

EOSIS,  iateeal) 

—  in  locomotor  ataxia,  213 

—  propagation  of  lésion  by  internai 

radicular  fibres,  214 

—  in  disseminated  sclerosis,  213,  215 

—  lésions  of  tbe  great  sympathetic, 

in,  226 

—  saturnine,  225 

—  rheumatic,  225 

—  traumatic,  225  . 

Anatomt,  topographie  microscopic,  6, 

227 
An^sthesia  in  ataxia,  14,  237 

—  in  painf  ni  paraplegia,  91 

—  in  spinal  hemiparaplegia,  106 

—  in  acute  central  myelitis,  160 

—  in  pachymeningitis,  212 
AoETA,  aneurisuis  of,  opening  iuto  the 

rachis,  76,  83,  92 
Artheitis,  dry,  of  tbe  racbis,  72 
Aetheopathies  of  ataxic  patients,  31, 

33,  49,  305 

—  frequency,  50 

—  epocb  of  appearance,  50,  53,  58 

—  clinical  f  acts,  51,  305 

—  température,  52,  53 

—  forms  and  symptoms,  54 

—  diagnosis,  diflerential,  55 

—  lésions  of  bones,  56,  307 

—  lésions  of  anterior  cornua,  60 

—  consécutive,  ou  lésions  of  the  peri- 

pberal  nerves,  58 

—  consécutive,    on     cérébral     hemi- 

plegia,  58. 


Aetheopathies,  consécutive,  on  trau- 
matic lésions  of  the  spinal  cord, 
58 

—  in  spinal  hemiplegia,  96,  114 
Ataxia,  iocomotoe,  progressive,  3 

—  anomalies,  4 

—  classic  description,  22 

—  cephalic  symptoms,  22,  28,  34,  44 

—  spinal  symptoms,  22 

—  lightning  pains,  23,  25,  306 

—  incoordination,  23 

—  paralytic  period,  24 

—  viscéral  symptoms,  28 

—  treatment,  61 

—  luxations,  patbological,  in,  305 

—  spontaneous  fractures,  307 

—  summary  of   symptoms,  236  {see 

An^sthesia  ;  Aetheopathies  ; 
Ateophy,  muscular  ;  Ceises, 
gastric  ;  Pains,  liglitning  ; 
Tabès  dorsalis  spasmodic) 

Athetosis,  390 

Ateophy,  muscular  progressive  pro- 
topathic, 9,  60,  144,  163, 
164 

—  individual,  of  muscles,  168 

—  functional  disorders,  160 

—  modes  of  invasion,  171 

—  course,  duration,  causes,  172 

—  lésions  of  spinal  cord,  173 

—  lésions  of  anterior  roots  and  peri- 

pheral  nerves,  176 

—  lésions  of  muscles,  178,  336 

—  lésions  of  bones,  313 

—  pathogeny  of,  315 

—  case,  327 
Ateophy,  deuteropathic,  91 

—  in  ataxia,  15 

—  in  hemiparaplegia,  107 

—  in  cérébral  hemiplegia,  207 
— •  in  acute  central  myelitis,  159 

—  in  pachymeningitis,  212 

—  in  gênerai  spinal  paralysis,  216 

—  in  infantile  paralysis,  131,  134 

—  in  cervical  paraplegia,  109 

—  consécutive  on  nerve-lesions,  256 

—  nervous,  progressive,  243 

—  partial,  of  brain,  280 


39  ( 


INDEX. 


Axis  cylinders  of  nerve-tubes,  tume- 

ftutioii  of,  317 
Aura,  motor,  266,  288 

B. 

Bakds,    exteenal,   of    tlie    posterior 

fascicles  of  the  cord,  15,  27     • 
Bladdee,  contraction  of  neok  of,  97 

—  paralysis,  98 

—  Budge's   theory   as   to   action   of 

nerve-sjstem    on   functions  of, 
98 

—  disorders    of,   in    compression    of 

cord,  116 

—  in  acide  spinal  paraJysis,  159 

—  in  acute  myelitis,  160 

—  in  urinary  paraplegias,  257 
BONES,  arrest  of  developnient  of,  in  in- 
fantile   paralysis,   132  {see  Ak- 

THEOPATHIES,  AtAXIAS) 

Eeain,  280  {see  Ateopht,  paetial) 

Beomide  of  potassium,  265,  273,  297 

BtTLBus  EACHiDicus,   334,   354,  360, 

368  {see  Coed,  Neeves,  Paea- 

liYSis,  glosso-kryugeal  ;    Scle- 

eosis) 

C. 

€ancee,  vertébral,  84,  88 
Caeies,  vertébral,  latent,  88,  91 
Cautérisation,   witli   actual  cautery, 

269 
Cells,  motor  nerve-  (tuméfaction  of), 

317 

—  in  myélites,  137,     156,    207  {see 

Ateopuy,    muscnlar  ;    Bulbus 

Coed,  Coenua) 
Centee,  genito-spinal,  254 
Cephalalgia    in    sypbilitic    cpilepsy, 

289 
Chorea,  common,  279 

—  symptomatic,  284  {see  Athetosis, 

HEMICnOEEA) 

Claw-hand,  171 

—  in  pachymeningitis,  212 

—  in    latéral    amyotropbic   sclcrosis, 

344 
CoLUMNS,  latéral,  devclopment  of,  182 
Concussion,  cérébral,  119 
Consanguinity,  172 
•CoNTEACTiLiTY,  ELECTEIC,  in  Saturnine 

and     rheumatic     amyotrophies, 

225 

—  in  protopatbic  muscnlar  atropby, 

170,  197 

—  in  acnte  central  myelitis,  160 

—  in  gênerai  spinal  paralysis,  216 

—  in  infantile  paralysis,  131 

—  in    parAplegia   from  compression, 

100.  112' 


Conteacture,  14,  21,  91,  96,  127,  171 
• —  in  hemichorea,  279,  281 

—  in  liemiparaplegia,  107 

—  in  piicbymeningitis,  212 

—  in  infantile  paralysis,  127 

—  in  latéral  sclerosis,  127 

—  in  spasmodic  tabès,  238,  244 
Coed,  spinal,  embryouic  development 

of,  181 

—  topographie   anatomy   of   67,   82, 

227      {see      Columns,     Goll, 
Turck) 

—  elementary  affections  of,  227 

—  bypertrophy  of,  165 

—  tumours  of,  67 

—  kystic  dilatation  of,  69 

—  ecbinococci  of,  70 

—  glioma  of,  68 

—  gumma  of,  69 

—  tubercles  of,  69 

—  compression  of  cord,  76 

—  transverse  myelitis,  78 

—  secondary  sclerosis,  78 

—  lésions  of,  in  cured  cases  of  Pott's 

disease,  80 

—  régénération  of  nerve-tubes,  81 

—  symptoms  of  compression  of,  81 

—  pseudo-neuralgias,  84 

—  disorders  of  motility,  96 

—  of  sensibility,  99 

—  abrupt   compression   of    cord    at 

ueck,  121 

—  lésions  of  lumbar  enlargement,  121 

—  of  tlie  cauda  equinn,  122 

—  traumatic  lésions,  161 

—  state   of    cord   in    pseudo-hyper- 

trophie paralysis,  378  {see 
Ataxia,  Atrophy,  Bulbus, 
Cells,  Cornua,  Paralysis 
Glosso-laryngeal,  and  In- 
fantile sclerosis,  &c.) 
Coenua,  anterior,  of  tlie  cord,  relations 
with  the  radicular  nerve- fila- 
ments, 16 

—  lésions  of  nerve-cells  of,  112,  155 

—  after  old  amputations,  157 

—  in  protopatbic  muscular  atrophy, 

174       {see       Artheopathies, 
Ataxia,  Atrophy,  muscular) 

—  posterior,  lésions  of,  11,  14 
CouGH,  in  compression  of  cord,  114 
Crises,  gastric,  29,  4-4,  115 

—  viscéral,  28,  48 
Cystitis,  252 

D. 
Déformations  in  muscular  atrophy, 
170 

—  in   latéral   amyotrophie    sclerosis, 

198  {see  Paealysis,  infantile) 


INDEX. 


3'J7 


Degkner ETIONS,  secoudary,  19, 21,  91., 

106,  205,  207,  24G 
DiPLOPiA,  35 
DisoRDERS  of  genito-urinarj'  organs  in 

ataxia,  28 
Dura  mater,  70,  83 

—  tumours  of,  70, 115  (see  Méninges) 
Dys^sthesia,  99,  246 
Dysphagia    iu    compression    of     the 

cord,  116 

—  in  glosso-laryugeal  paralysis,  364 
Dyspnœa  in  compression  of  tlie  cord, 

114 

—  in  glosso-laryngeal  paralysis,  364, 

366 

E. 

Eae,  lésions  of,  in  Ménière's   vertigo» 

263,  266 
Embolism   of    the    arteria    centralis 

retince,  41 
Épilepsy  in  compression  of  the  cord, 

116 

—  partial,  281,  287 

—  partial,  of  syphilitic  origin,287 

—  cephalalgia,  289 

—  description  of  fits,  288,  294 

—  treatment,  290,  293,  298 

—  spinal,  116, 127,  202,  236,  245 
Epileptic    acme,    status    epilepticus, 

288 
EsCHARS  in  spinal  hemiplegia,  100 

—  in  lésions  of  cauda  equina,  122 

—  in  central  myelitis,  160 

—  in   paraplegias  from   compression, 

107 

—  in  urinary  paraplegias,  253,  257 

G. 
Glioma,  68,  167 
Glycosttria,     with     lésion    of     optic 

nerves,  41 
GoLi,  developmeut  of  GoU's  columns, 

180  (see  Sclerosis) 

H. 

Hallucination,  motor,  262,  267 
H^MATOMA,  69,  71,  210 
H^matomyelia,  69,  128, 161,  322 
Hemian^sthesia  of  cérébral   origin, 
276,  282,  284 

—  decussated,  theory,  103 

—  hysterical,  105,  264 

—  spinal,   with   crossed    ansestbesia, 

"  101,  103,  247 
Hbmichorea,  post-hemiplegic,  275,280 

—  prœ-hemiplegic,  283 
Hemiparaplegia,  spinal,  with  crossed 

anaîsthesia,  102,  247 


Hemiplegia,  cérébral,  with  contrac- 
ture, 199 

—  spasuiodif,  280 

—  spinal,  101 
HffiMORRHAGE,  intra-encephalic,  281 
Heredity,  172,  196 

Hiccup  in  spinal  paralysis,  116 
Hydeomyelitis,  166 
Hydeotuerapy,  297 
Hyper^sthesia,  170,  294  {see  Hemi- 

paeaplegia) 
Hypeetrophy  of  the  cord,  209 
Hypochondeia,  27, 170 
Hysteria,  ovariau,  277 


Incoordination,  motor,  23 
Inhibitory  phenomeua,  259 


Kysts,  hydatid,  69,  71,  83 

L. 

Lipomatosis,  geueralised,  217, 221, 224 

—  interstitiai,  179 

—  luxuriant,  179,  224 
Luxations,  305  (see  Ataxia) 

M. 
Mediastinum,  dyspnœa  in  tumours  of, 

376 
Méninges,  spinal,  67,  208 

—  tumours  of,  70  (y.  Dura  matée) 
Meningitis  of  base  of  brain,  42 

—  spinal,  posterior,  6 
MoNOPLEGiA,   transient,  of   syphilitic 

origin,  294 
Muscles,  lésions  of,  iu  glosso-lai'vnceal 
paralysis,  221,  378 

—  pseudo-hypertrophie,      368       {see 

Amyoteophies,  Ateophy) 
Myelitis,  central,  127,  154,  159 

—  consécutive,  on  diseases  of  ui-inary 

passages,  252,  257 

—  partial,  lightning  pains,  28,  252 
— ■  spontaneous,  96 

—  traiisverse,  78,  94,  245,  253 

—  from    compression,   97,   235    {see 

CoED, spinal) 

—  tuméfaction  of   motor  nerve-cells 

and  of  axis  cylinders  in  certain 
cases  of,   317    {see   Abtheopa- 

TUIES) 

Myopathies,  spinal,  125 

N. 
Nepheitis,  252 

Neeves,  lésions  consécutive  on  section 
of,  158 


398 


INDEX. 


NeeteS,  bulbar  lésions  of ,  34  {see  Amt- 

OTKOPHIES,  PAEALYSIs) 

cérébral,  atrophy  of,  6,  34 

—  optic,  atropliy  of,  38 
rrrey  induration  of,  39 

—  atrophy  of  optic  papilla,  35 

—  diagnosis,  89  {see  Neuritis) 

—  phrenic,  335 

—  peripberal,  lésions  of,  58,  219,  221, 

335 

—  sciatic,  avulsion  of,  215 
Neueitis  of  optic  nerves,  35,  41 

—  pai'euchymatous,  38 

—  of  sciatic  nerve  in  urinary  para- 

plegias,  260 
Neueoglia.  138,  142,  338,  352,  360, 
370 

NEtTEO-EETINITIS,  41 

Nuteition,  disorders  of,  in  paraplegias 
from  compression  {see  Amyo- 
TKOpniES,  Muscles,  &c.) 

O. 

Object-leSSONS,  in  amyotropbics,  275 
OSSEOUS  SYSTEM,  132  (see  BONES) 

P. 

Pachymenin&itis,  71 

—  caseous,  73,  94,  109 

—  cervical    hypertrophie,    92,     165, 

209,  212 

—  gummatous,  299 

Pain,  23, 197  ;  boring,  25  ;  lancinating, 
25  ;  constrictive,  26  ;  gênerai 
characters,  26  ;  anatoinical  sub- 
stratum,  27-;  vesical  and  ureth- 
ral,  28,48;  rectal,  29,  48 

PaealysiS  agitans,  284 

—  from  compression,  169 

—  gênerai      progressive,     lightning 

pains,  27 

—  lésions  of  optic  papilla,  34 

—  gênerai  spinal,  gastric  crises,  29, 

128,  215 

—  glosso-laryngeal,  128,  201,  363 

—  lésions  of  muscles,  367,  368 

—  of  motor  nerve-cells,  126,  370 

—  pseudo-liypertrophic,  221 

—  anatomioal  state  of  muscles,  378 

—  spinal,  acute,  of  adults,  144 

spinal,  acute,  of  children,  129,  156 

—  modes  of  invasion,  129 

—  chillitig  of  limbs,  133 

—  déformations,  133 

—  lésions  of  muscles,  134 
— ■  lésions  of  cord,  137 

Yaeaplegia,  alcoholic,  28 

—  cervical,  108,  199 

—  of  cancerous  patients,  74,  89 


Paeaplegia  from  compression,  65 

—  consécutive,  on  intestinal  lésions, 

259 

—  consécutive,  on  utérine  lésions,  260 

—  reflex,  257 

—  saturnine,  225 

—  urinary,  250 
Pemphigus,  213 
Prolongations,  nerve,  19 

—  protoplasmic,  19 

PSEUDO-NEURALGIAS,  76 

—  in  cervical  paralysis,  109 
Psoriasis,  palmar,  syphilitic,  292 
PuLSE,  retardation  and  frequency  of, 

in  gastric  crises,  30,  49 

—  frequency  in  ataxia,  49 

—  in  glosso-laryngeal  paralysis,  366 

—  retardation  in   lésions  of  cervical 

cord,  108,117 

—  permanent  slow,  118 

—  probable  lésions  of  bulbus,  118 

—  in  latéral  sclerosis,  357  (see   Ra- 

CHIS) 

Pfpils,  113 
Pyelo-nepheitis,  252 
Pyeamids,  anterior,  sclerosis  of,   355 
(see  CoED,  Sclerosis) 

Q. 

QUITEEING,  FiBEILLAEY,  170 

R. 

Rachis,  abscess  of  cellular  tissue  of,  71 

—  Pott's  disease  of,  72 
~  cancer  of,  74 

—  hydatid  kysts  of,  71 

—  narrowing  of, — witli    slow    puise 

and  cpilepsy,  71,  116,  117,  120 
(see  Aethritis) 
Roots,  anterior,  of  nerve,  347, 351, 359 

—  posterior,  5,  83,  347,  351 
Ramollissement  of  brain,  281 
Rectum,  disorders  of,  in  compression 

of  cord,  29,  48  (see  Pains) 
Reflex,  exaltation  ofphenomena,  111, 

127,  130 
Rénal  disorders,  252  (5ee  Paraplegias, 

urinary) 
Retino-ciioroiditis,  syphilitic,  41 
Rétrécissement  of  urethra,  252 

S. 

Saecoma,  angiolithic,  70 
Satyriasis,  48 
Sclerosis,  7,  341 

—  of  extornal  bands,  11,  12,  13,  27 

—  of  Goll's  columns,  10,  11,  27 

—  of  latéral  cohimns,  7,  21,  139,  180 

—  latéral  amyotrophic,  167,  180,  341 

—  lésions  of  latéral  columns,  180 


INDEX. 


399 


SCLEEOSIS  of  grey  substance,  167,  353 

—  of  auterior  roots,  177 

—  of  peripheral  neryes,  177 

—  of  muscles,  178,  190,  349 

—  symptoms,  192 

—  évolution,  197 

—  causes,  196 

—  atrophy,  en  niasse,  197 

—  rigidity,  200 

—  bulbar  phonomena,  201,  341 ,  355 

—  patbological  pliysiology,  203 

—  diagnosis   from    spasmodic  tabès, 

235,  247 

—  two  cases,  341 

—  dissemiiiated,  7,  27,  247 

—  lightning  pains,  24,  27,  170,  197, 

248 

—  of  posterior  columns,  3 

—  by  extension  of  latéral  sclerosis,  13 
Sensations,   retardation  in  transmis- 
sion of,  99 

—  associated,  100 
Steatosis,  pbysiological,  184 

SUIPHATE  OF  QUININE,  272,  274. 

Stmpathetic,  lésions  of  great,  226, 388 
Symptoms,  récurrent  or  rétrograde,  19 
Syphilis,  286  (see  Cord,  Epilepsy, 

MoNOPiEGiA,  Psoriasis,  Reti- 

nochoeoiditis) 
Sybingomyelia,  166 


Tabès  doesalis,  234 

—  spasmodic,  233,  245 

—  comparison  with  ataxic  tàbes,  235 

—  gait,  238 

—  causes,  243 

—  diagnosis,  244,  248 

—  course,  243 

—  duration,  243 

—  seat,  242 

—  treatment,  243 

—  trépidation,  236,  240 


Tabetic  symptoms,  3,  5 
Tempebatube,  gastric  crises,  30 

—  ligbtning  pains,  52 

—  arthropathics,  52,  53 

—  spinal  hemiparaplegia,  103 

—  acute    spinal   paralysis   of  adults, 

147 

—  glosso  -  laryngeal    paralysis,    366, 

376 
Tenesmtjs,  29,  48 
Tepheo-myelitis,     acute     parenchy- 

matous,  158 

—  chronic,  173 
Trembiing,  senile,  284 
Trépidation   in  transverse    myelitis, 

245 

—  of  hémiplégie   patients,   236  {see 

Tabès  doesalis) 
TUBERCLES  of  vertcbrœ,  281  {see  Coed) 
Tumoues,    cérébral,    {see   Cord,   mé- 
ninges) 
TuRCK,  development  of  columns  of,  181 

U. 

Urethra,  Urine,  Uteetts  {see  Paea- 
PLEGiAS,  Rétrécissement,  Dis- 
orders) 


VeSSELS,   lésions,   in   latéral  sclerosis, 

331 
Veetigo,  epileptic,  265 

—  gastric,  265 

—  of  Méuière,  261 

VoMiTiNG  in  ataxia  {see  Crises,  gas- 
tric) 

—  in  Ménière's  vertigo,  262,  269 


Zona,  91,  151 

Zones,    radicnlar,   anterior,    posterior, 
181,  182,  183 


PEINTED   BY  J.   E.   ADLARD,  BARTHOLOMEW   CLOSE. 


PLATE   I\  VOL.  I. 

DISSEMINATED  SCLEROSIS  (ENCEPHALON). 

EiG.  I, — Base  of  the  brain. 

a.  Patches  of  sclerosis  disseminated  aloug  tlie  olfactory  nerves, 
h.  Islets  of  sclerosis  along  the  optic  nerves, 
U .  Healthy  portion  of  one  of  the  optic  nerves. 

c.  Islets  of  sclerosis  on  the  left  crus  cerebri. 

d.  Patches  of  sclerosis  disseminated  over  the  protuberantia,  some  being 

superfîcial,  others  deep-seated  ;  surface  somewhat  depressed  at  the 
patches.   The  nerves  emerging  from  the  protuberantia  appear  healthy. 

e.  Patches  of  sclerosis  irregularly  distributed  over  différent  parts  of  the 

bulbus  rachidicus   and  medulla  oblougata — anterior  pyramids  (es- 

pecially  the  right)  olivary  body,  antero-lateral  column. 
e'.  Healthy  portions  of  the  medulla  oblongata. 
/.  The  terminal  transverse  section^    showing  how  deeply  the    scierons 

lésion  has  penetrated  the  substance  of  the  cord  at  this  level,  and  the 

irregularity  of  its  distribution. 
/'.  Some  healthy  remnants.    The  nerves  emerging  from  the  bulbus  appear 

healthy. 

'Svi.  2.— Horizontal  section  of  the  cerehellum,  made  so  that  the  two 'parts  thus 
symmetrically  divided  may  be  readily  fulded  together. 
x.y.  Intersection  line  of  the  horizontal  and  vertical  planes,  formed  by 
the  section. 

a.  Patches  of  sclerosis  disseminated  in  the  white  substance. 

b.  Sclerous  patch  invading  the  corpus  rhoniboideum. 

c.  Patches  of  sclerosis  wbich  hâve  been  divided  almost  symmetrically  in 

two. 

d.  Blood-vessels  plaiuly  visible  in  the  midst  of  the  sclerous  nodules. 

e  {rectèf).  Blood-vessels  in  the  white  substance  becomiug  more  évident 
on  exposure  of  the  section  to  the  atmosphère.  Scattered  red  dots, 
very  plainly  marked. 

!FiG,  3. — Portion  of  base  of  the  brain. 

a.  Olfactory  nerves  apparently  healthy. 

b.  Islets  of  sclerosis  in  the  optic  nerves. 

c.  Islets  of  sclerosis  in  tlie  crura  cerebri. 

d.  Patches  of  sclerosis,  disseminated  over  différent  parts  of  the  protu- 

berantia, some  superfîcial,  others  decp-seated.  Surface  slightiy  de- 
pressed at  thèse  points.  The  nerves  emerging  from  the  pons  appear 
healthy. 

e.  Patches  and  islets  of  sclerosis  irregularly  distributed  over  différent 

parts  of  the  bulbus  rachidicus  and  medulla  oblongata, — anterior 
pyramids  (completely  invaded),  olivary  bodies  (incompletely). 

,/.  The  terminal  section  displays  the  depth  to  which  the  sclerous  lésion 
has  penetrated  the  cord  itself,  and  the  irregularity  of  tlie  indura- 
tion.    The  nerves  emerging  from  the  bulbus  appear  healthy. 

g.  Sclerosis  beginning  in  the  constituent  tissue  of  the  posterior  locus 
perforatus. 

*  This  and  the  three  succeeding  plates  hâve  been  taken  from  the  Note 
(already  quoted),  by  M.  Henri  Liouville,  Director  of  the  Laboratory,  at 
the  Hôtel  Dieu. 


'l.  1 


T.      tj      —i    ns  r^        a)      a) 


w       f    *»• 


-^  TS  l» 


G.Feliizr   ei  Oyon.  oclvuii  cUl. 


W«st,  Nfcv/nwuv  i  0?  irhi'OMi.US- 


PLATE   II,  VOL.  I. 

DISSEMINATED  SCLEROSIS  (CEREBRUM). 

FiG.  1.— Horizontal  sedioti  of  the  cerebrum,  displayhig  the  idels  of  selerosis  in 
différent  régions  {both  of  whiie  substance  and  grey  substance). 

a.  Patches  and  islets  of  selerosis  in  the  anterior  régions  (anterior  com- 
missure, part  adjacent  to  third  ventricle). 

h.  Scierons  patches  invading  the  anterior  parts  of  the  margin  of  the 
latéral  ventricles  (ventricular  patches). 

c.  Extension  of  scierons  islets  to  the  posterior  extremities  of  the  latéral 

ventricles  (ventricular  patches) . 

d.  Islets  of  selerosis,  irregularly  disseminated  in  the  white  substance  of 

the  posterior  cérébral  régions  ;  some  of  thern  are  very  deep-seated. 

e.  Blood-vessels  plainly  visible  in  the  midst  of  the  zones  of  selerosis. 

f.  Blood-vessels  in  the  apparently  healthy  white  substance  becoming  more 

and  more  évident  on  exposure  of  the  section  to  the  air. 

FiG.  2. — Ânother  horizontal  section  of  the  same  cerebrum,  displayitig  the  islets 
of  selerosis  in  other  régions  (both  of  white  and  grey  substances). 

a.  Patches  and  islets  of  selerosis  in  the  anterior  régions  (anterior  com- 

missure). 

b.  Scierons  patches  in  the  anterior  parts  of  the  latéral  ventricles. 

c.  Patches  of  selerosis  in  the  grey  substance  of   the  intra-ventricular 

nucleus  of  the  right  corpus  striatum.     (They  are  multiple,  divided 
by  healthy  spaces  ;  some  are  deep-seated). 

c'.  Extension  of  the  sclerotic  islets  to  the  posterior  extremities  of  the 
latéral  ventricles. 

d.  Islets  of  selerosis  disseminated  irregularly  through  the  white  substance 

of  the  posterior  cérébral  régions.     Some  are  very  deep. 

e.  Blood-vessels,  in  apparently  healthy  parts  of  the  white  substance,  be- 

coming more  and  more  évident  on  exposure  of  the  section  to  the 
atmosphère.    (Very  marked  dottiug.) 


PI.  n. 


.,/<'*"'**è^^  > 


Ix^ 


Oy       •'O 


P.:  Ci 


cV   aii^  nA>t.a.ei 


V/esv  N^cwvtux^  de  C  *?  ckrcv*x  hJth 


PLATE   III,  VOL.  I. 
DISSEMINATED  SCLEROSIS  (SPINAL  CORD). 

FiG.  I. — Posterior  aspect  of  the  spinal  cord  {ihe  dura  mater  is  divided  and 
throicn  back  at  either  si  de), 
s.  Patches  and  islets   of  sclerosis,  irregularly  disseminated,  varions  in 
form  and  dimensions,  irrcgular,  isolated  or  partially  united  by  connec- 
tions visible  on  the  surface.    They  predominate  hère,  especially  in  the 
dorsal  région. 
V.  Very  markcd  mcningeal  (pia  mater)  vascularisation,  which  prevents  the 
spécial  vascularisation  of  the  sclerotic  patches  from  being  discemed. 

FiG.  2. — Anterior  aspect  of  the  spinal  cord  {the  dura  mater,  divided  from  top  to 

bottom,  is  thrown  back  at  either  side). 

s.  Patches  and  islets  of  sclerosis,  irregularly  disseminated,  with  unsymme- 

trical  borders  ;  isolated  or  united  by  connections  visible  on  the  surface. 

V.  Mcningeal  (pia  mater)  vascularisation,  predominating  and  preventing 

the  spécial  vascularisation  of  the  sclerotic  zones  from  being  discemed. 

PiG.  3. — Horizontal  sections  taken  from  différent  levels  of  the  spinal  cord  and 

displaying,  in  cvery  région,  the  depth  of  the  sclerotic  islets,  their  un- 

eqval  distrilnition,  and  the  irreyulurity  of  their  disposition,  both  in 

the  white  substance  lohere  they  predominate,  and  in  ihe  grey  substance. 

Ail  thèse  sections  represent  the  fresh  and  unprepared  condition  of  the 

cord.     They  are  seen  from  above,  the  cord  being  vertical. 
a.  Anterior  margin. 
p.  Posterior  margin. 
s.  Islets  of  sclerosis. 

The  scierons  patches  are  represented  with  their  natural  hue,  which  con- 
trasts  so  plainly  with  the  white  substance  and  even  with  the  grey 
central  substance. 
No.  I. — Superior  portion  of  tlie  cervical  région  immediately  beneath  the 

bulbus  racbidicus, 
„    2. — Middle  portion  of  cervical  enlargement. 
„    3. — Inferior  portion  of  cervical  enlargement. 
„    4. — Superior  portion  of  dorsal  région. 

„    5. — Two  centimètres  (=  o"78  inch)  lower,  superior  dorsal  région. 
„    6. — One  and  a  half  centimotres  (=  0-58  inch)  lower,  superior  dorsal 

région. 
„    7. — Two  centimètres  lower,  junction  of  the  superior  ihird  with  the 

middle  third  of  spinal  cord. 
„    8. — One  centimètre  and  a  half  lower,  dorsal  région. 
„    9. — One  centimètre  and  a  half  lower. 
„  10. — Two  centimètres  lower,  middle  of  dorsal  région. 
„  II. — One  centimètre  (=  o"39  inch)  lower. 
„  12. — One  centimètre  and  a  half  lower. 
„  13. — Three  centinictrcs  (=  l'iS  inch)  lower. 
„  14. — Superior  portion  of  dorso-lumbar  enlargement. 
,,  15. — Middle  of  dorso-lumbar  enlarg;ement. 
„  16  and  17. — Terminal  cône. 


PI.  m. 


Posterior   SLspect. 

-M 


Anten  or    a-spect . 


Pi  g.    i. 

G.Peiiiyer  oui  yunt  ditl 


Tig    2. 


PLATE   IV,  VOL.  I. 

DISSEMINATED  SCLEROSIS  (SPINAL  CORD). 

i'iG.  I. — Posterior  aspect  of  llie  fpiiial  cord  {the  dlvided  dura  mater  is  thrown 
haek  at  either  sidé). 

c.  Patelles  of  sclerosis,  dissemicated  irregularij. 

V.  Meningeal  (pia  mater)  vascularisation,  predomiuating  and  preveuting 
the  vaseularity  of  the  sclerotic  patches  from  being  discerned. 

FiG.  2. — Anterior  aspect  of  the  spinal  cord  (the  dlvided  dura  mater  is  thrown 
hack  at  either  side). 

s.  Patehes  aud  islets  of  sclerosis,  disseraiuated  irregularly. 
V.  Meningeal  (pia  mater)  vaseularisaiioa. 

TlG.  3. — Horizontal  sections  ofthe  cord,  takenfrom  différent  levels,  and  display- 
ing  in  every  région  the  depth  of  the  sclerotic  islets,  and  their  unequal 
and  irregular  distribution,  both  in  the  columns  ofthe  white  substance, 
lohere  they  predominate,  and  in  the  grey  substance. 

(Ail  thèse  sections  were  taken  from  the  fresh  unprepared  cord.) 

a.  Anterior  raargin. 

b,  Posterior  margin. 
s.  Sclerosis. 

(The  sclerosed  portions  are  represented  in  their  natural  tint,  which  con- 
trasts  plainly  with  the  white  substance,  and  even  with  the  central 
grey  substance.) 
No.  I. — Superior  portion  of  cervical  eulargemeut. 

2. — One  centimètre  and  a  half  (=  o"58  inch)  lower. 

3. — Two  centimètres  (=  o'78  inch)  lower  (end  of  cervical  eularge- 

ment). 
4. — Two  centimètres  lower  (superior  portion  of  dorsal  région). 
5. — One  centimètre  and  a  half  lower. 
6. — Two  centimètres  lower. 
7. — Three  centimètres  (=  i*i8  inch)  lower. 
8. — One  centimètre  aud  a  half  lower. 
9. — Two  centimètres  lower. 
10. — A  little  more  than  one  centimètre  (0*39  inch)  lower  ;  the  cord 

hère  is  healthy,  or  nearly  so. 
1 1 . — One  centimètre  above  the  dorso-lumbar  enlargement. 
12. — Middle  of  the  dorso-lumbar  enlargement. 
13. — A  little  bclow  the  begiuuing  of  the  terminal  coue. 
„  14. — Filum  terminale,  com))Ietcly  invadcd  by  sclerosis. 


Posi'erior  aspect- 


S^c 


t-. 


■b 


-S       4- 


m 


Tia.  3 


13 


Jig.2. 

West  Newyna*vSc  C?  chrô    Util 


PLATE  V,  YOL.  I. 

HYSTERICAL  ISCHURIA. 

The  blue  Une  indicates  the  quantity  of  urine  passed  iu  the  twenty-four  liours, 
and  the  red  line''i\iQ  amount  of  vomited  maiter.  [The  daily  average  is  given 
at  foot  of  the  Table.] 


Plaie    V. 


c.                                  "2 

f*                                ^s 

'" 

x*                                    > 

N                                                         ^ 

o                                         ^ 

M                                     •'s. 

Ol         l_ 

^N^ 

<^                                   --' 

00                                          _i 

^                         -^^^ 

^ 

^> 

.y 

<C                                                       -!^ 

-^                                                  X*^ 

"' 

^2                       -.^ 

T^                        -                    -^ 

^                     N^ 

3                       7 

o                   -.L 

■^                      , 

î:                              * 

^ 

^ 

'■' 

o                                l 

^                                 \ 

®                                J 

"^                              7 

Où                                    V 

00                                  ^ 

1-,                             W 

■^                             ^^ 

> 

^Z' 

^^ 

4^ 

^v 

03 

^                                 -£=" 

^s^ 

^ 

^                                 "^. 

^                        _1_              S 

>^ 

OJ 

CO                                         >^ 

^ 

M                                     ^^ 

^^ 

< 

y 

^^^ 

^'^ 

^^^ 

^-■^ 

■^i- 

^                             ^-•- 

L                                              J 

o                   *^' 

CO                                 ^. 

L 

05                                 \ 

\ 

N                                          '^u 

T 

00                              S^ 

4 

W                                         y^ 

5 

■>                                   -.^ 

V 

(M 

t 

U> 

l 

■^^                         ^S^      ...._ 

5 

U5                                               -^^ 

^^ 

N                              ^^     -i 

2 

■*                      -ld_ 

^ 

s                       "-^ 

5^ 

"•»•                         ^ 

0»                                                             ^*« 

Z 

l 

?«                   ^^ 

\ 

\^ 

y. 

t 

4j' 

J 

3 

a                         1 

T 

Hi 

a>                                          j 

^ 

^^^                     t 

00 

%^             4 

, 

th 

^^                 ^r 

00 

■^                         ^ 

^                                                  ..     1 

t 

^                                                         J 

4 

^ 

'H 

O 
O 
O 


O 
Lfi 

OS 

(M 

4) 

ri 


I  I  I  I  I  I 

OOOOOO 

O  in  <i<  CO  <N  ■H 


West ,  "Newm  an  %.  Co .  Sot.  Tith 


PLxiTE  VI,  VOL.  I. 

HYSTETIICAL  ISCHTJRIA. 

The  hlue  Une  iudicates  the  quautity  of  urine  passed  in  the  twenty-four  liours, 
and  the  red  Une  that  of  the  vomited  matter. 

The  small  red  sqicares,  placed  immediately  beneath  certain  dates,  dénote  the 
days  of  analysis. 

[The  daily  average  is  given  at  foot  of  the  Table.] 


Plaie     Yl. 


C£>    U-iw 

^  F 

>^  L  k. 

F 

■^       Li^                                                          ^ 

^       1    ^ 

M     U^ 

^  F 

co   t^ 

^  F 

^  C-> 

-'  r*^ 

"•s^ 

S. 

'  ' 

%^ 

<X) 

^ 

r, 

-I-' 

^ .-  ■^'^ 

o 

w         -.- 

n 

rH              4 

^              ^^^. 

§                    ^;?- 

0^  -^            -«^^ 

0.                            -<>^ 

g^                       ^* 

<N                                               _,--* 

t-                                        e'"^'' 

<N                                    '' 

^^^ 

«i 

^^ 

N                          ^ 

^s. 

1^                                    ^ 

S 

^                                    ^ 

■*                                         ^ 

M                                         ;» 

«                -^^'' 

^                ^"^ 

eg                         "*'»'^ 

(M                                         ^ 

—1 

ca                            -j 

°                        ^C 

'^                            i 

Oî                                   \ 

s 

^^^ 

00                               ^v 

^^ 

^> 

<s^ 

t-             ^"^ 

■N^ 

s            *«^^ 

^                ^-^^ 

v-l                                                             'I 

lO                                                                                                                 L 

^"^^ 

'"'                       '*"*'*-. 

(f.                                       *"  «»  ^ 

». 

Cl 

N      it 

z                ( 

. 

Z                        j 

>^ 

V 

o                              ^-^ 

t 

^                    .' 

m                  .^ 

Oi                                     -n^^ 

(«                                                         "^^ 

*                                                              ? 

^.                             "                                 H 

1 

y 

'^ 

,o                                             i 

y 

fl 

tC                                  ■*; 

^s 

ii 

^!^ 

^                               -.^ 

^                             "*s^ 

A^                                        \ 

'                                         ? 

z 

•«'^ 

7 

C4                                             *' 

\ 

"          it     -.<î 

^v 

oo 

' 

cO 


U      ^0 


^ 


CM  •*  • 

o  S 

.   o 


^ 


^1 


P>° 


?4 

;3 


^ 


1 1 1 1  )  I 

OOQOOO 


PLATE  VII,  VOL.  I. 

HYSTEEICAL  ISOHURIA. 

The  bine  Une  indicates  the  quautity  of  urine  passée!  in  the  tweut.y-four  lionrs, 
and  the  red  Une  that  of  tlie  vomited  matter, 

The  small  red  squares,  placed  immediately  beneath  certain  dates,  dcnolc  tho 
days  of  analysis. 

[The  daily  average  is  given  at  foot  of  the  Table.] 


Plate    VIL 


1 
o 

1 
O 

1 

o 

1 
o 

1  1  1 

S9° 

o 

o 

o 

o 

N'-i 

o 

Ifl 

o 

Ift 

04 

^ 

w 

Wës  t ,  'Ne  vmû.a.-a. 

S^Co.  clir  litk . 


PLATE  I,  VOL.   IL 

SCLEROSIS  OF  THE  POSTERIOR  COLUMNS. 

ElG.  I  (left  side). — Transverse  section  of  tke  spinal  cord  at  tJie  sixth  dorsal 
vertebra. 

a,  a.  Small  nodules  of  sclerosis,  situated  in  the  external  ribands  of  the 

posterior  columns. 

b.  Sclerosis  of  the  interraediate  column.  ** 

ElG.  I  (right  side). — Transverse  section  of  the  cord  in  the  cervical  région. 

«,  a.  External  ribauds  of  the  posterior  columns,  not  exhibiting  any 
trace  of  sclerosis. 

FiG.  2  (left  side). — Transverse  section  of  the  cord  in  the  cervical  région. 

The  external  ribands,  a,  a,  the  médian  column,  b,  the  posterior 
cornua,  including  the  point  of  émergence  of  the  posterior  roots,  c, 
are  wholiy  invaded  by  sclerosis. 

FiG.  2  (right  side). — Section  of  the  dorsal  région  of  the  cord. 

The  sclerosis  has  invaded  the  same  parts  as  in  the  cervical  enlargement. 

FiG.  3  (left  side). — Tr ans  verse  section  of  the  cord  in  the  inferior  portion  of  the 
dorsal  région. 

a,  a.  Islets  of ,  sclerosis,  situated  in  the  external  ribands  of  the  posterior 

columns  and  connected  with  the  émergence  points  of  the  posterior 
roots,  c. 

b.  Small  sclerous  islet,  situated  immediately  behind  the  posterior  com- 

missure. 

FiG.  3  (right  side). — Transverse  section  of  the  cord  in  the  middle  of  the  cervical 


«,  a.  External  ribands  invaded  by  sclerosis. 
b.  Médian  column  remaiuing:  intact. 


Vol.  II.   PI.  I. 


Fiq-    1 


n     '  \f      ! 


Fig.   2 


a      -fa 


a         "b 


Fxg.   3 


.^r/r 


i// 


*iv 


J 


a  >, 


Wsst  Newnuw.  &  C'  cHr,Utà 


PLATE   II,  VOL.   II. 

POTT'S   DISEÂSE;   PARAPLEGIA. 

FiG.  I. —  fFMte  substance  of  the  cord  at  the  compressed  part  in  a  completely 
paraplégie  subject. 

a.  Trabeculse  of  sclerosis. 

b.  Nuclei  disseminated  iu  the  sclerous  tissue. 

c.  Section  of  a  vessel  wliose  sheath  is  continuous  with  the  sclerous  tissue. 

d.  Altered  nerve-tubes. 

e.  Schwann's  sbeath,  filled  with  granular  corpuscles, /. 

ff.  Nerve-tubes,  which  hâve  undergone  considérable  dilatation  and  défor- 
mation. 
h.  Axis-cylinder,  driveu  back  on  the  latéral  parts. 
ÏIG.  2. —  White  substance  of  the  cord  in  a  subject  who  had  been  cured  of  para- 
plegia,  and  who  died  of  an  intercurrent  disorder. 
a.  Sclerous  tissue. 

h.  c.  Regenerated  nerve-tubes  :  some,  b,  are  of  the  normal  size  ;  others, 
c,  are  very  much  smaller. 
PlG.  3. — Externat  pachymeningitis  :    lo7igitudinal  section  of  dura  mater  com- 
mencing  to  thicken. 

a.  Healthy  internai  portion. 

b.  Internai  portion  presenting  clusters  of  nuclei  in  the  intervais  of  the 

fibre  fascicles. 

d.  Section  of  vessel. 

e.  Section  of  vegetating  tissue. 

f.  Eléments  of  new  formation  :  nuclei,  fusiform  cells  and  corpuscles. 

g.  Looped  or  wavy  capillaries. 

h.  Stratum  deyoid  of  vessels,  and  formed  of  caseous  éléments. 


Vol.  IL.  PL  n. 


Fia.  2. 


West,New.na«,  &  C"  litK 


PLATE  III,   YOL.   II. 

POTT'S  DISEASE;  PARAPLEGIA. 

ElG.  I. —  'Examinatio7i  of  conl  in  successive  sections  in  a  case  of  dorsal  Pott's 
diseuse.    Secondary  degenerations. 

d.  Compressed  point. 

d'.  Same,  more  highly  magnified. 
c.  Dorsal  région.    Latéral  and  posterior  sclerosis. 
b.  Inferior  cervical  région.     Sclerosis  confined  to  GoU's  columns. 
.     a.  Cervical  enlargement,  id. 

e.  Inferior  dorsal  région.    Diffuse  sclerosis  of  latéral  columns. 
/.  Lumbar  région. 

g,  Lumbar  enlargement. 

FiG.  2. — Section  in  the  cervical  région  in  a  case  of  dorsal  Potfs  diseuse.     Annu- 
lar  sclerosis,  especially  manifest  towards  the  posterior  roots. 


Fiq.    1. 


\ol.II.  Pl.III. 


d 


d' 


W^m^^^'-^ 


Fiq.     2. 


West  J^ewyi\jxrh &C°  oki-  UtK . 


PLATE  IV,  VOL.  II. 

SYMMEÏRICAL    SCLEROSIS    OF    THE    ANTERO-LATERAL 
COLUMNS. 

FiG.  I.  Transverse  section  of  the  bulbus  racJiidicus  on  a  level  with  the  decus- 
sation  of  the  pyramids. 
a,  a.  Reticulated  formation  of  Deiters  and  latéral  columns. 
l.    Anterior  pyramids. 
c.  c.  Auterior  cornua  of  grey  substance. 
e.    Decussation  of  tlie  pyramids. 
p,  p.  Posterior  cornua. 

FlG..  2.  Transverse  section  of  the  spinal  cord  in  the  upper  part  of  the  cervical 
oïlargement. 

a,  a.  Latei-al  columns. 

h,  b.  Anterior  columns. 

c,  c.  Anterior  cornua. 

p.p.  Posterior  cornua. 
ElG.  3.  Transverse  section  of  the  spinal  cord  in  the  lower  part  of  the   cervical 


a,  a,  c,  c,  p,  p.  As  in  the  preceding  figure. 

f,f.  Foci  of  disintegration  occupying  différent  points  of  the  anterior  grey 
substance. 
ElG.  4.  Represents  the  différent  phases  of  pigmentary  degeneration  of  the  cell 
of  anterior  cormta. 
a.    Normal  cell. 
è,  c,  d.  Degeuerated  cells. 


Vol.  IT,  PI.  IV. 


^■MIL'JM^ 


West  Newnwn.  &  C?  chr  UiJv. 


PLATE  V,    VOL.   IL 

SYMMETRICAL   SCLEROSIS  OP  THE  ANTERO-LATERAL 
COLUMNS. 

FiG.  I . — Transverse  section  of  the  spinal  cord  in  the  middle  'part  of  tJie  dorsal 

région. 

a,  a.  Latéral  columns. 

c,  c.  Anterior  cornua. 

p,  p.  Posterior  cornua. 

ElG.  2. — Transverse  section  of  the  spinal  cord  in  the  middle  part  of  the  lumhar 
enlargement. 
a,  a.  Latéral  columns. 
c,  c.  Anterior  cornua. 
p,  p.  Posterior  cornua. 


Fiq-    1 


Yol.ir.  PLAT 


l[,U^\>^ 


Fiq       2. 


West  Vlewma^,  &  C?  chy.  lilh 


I 


PLATE  YI,  VOL.  II. 

LOCOMOTOll  ATAXIA. 

ïhis  engraving,  from  a  drawing  by  M.  Richer,  Ilouse-Surgeon,  represents 
the  patient  Cott — ,  whose  case  is  related  ia  Appendix  I,  p.  305. 


Vol.  II.  PL  VI. 


West  Nevjman  &  6"?  lliii 


PLATE  VII,  VOL  II. 

PROTOPATHIC  MUSCULAR  ATROPHY. 

FiG.  I. — Transverse  section  of  the  spinal  cord  in  the  cervical  région. 
A.  Anterior  radicular  zone,  sclerosed. 
C.  Anterior  cornu,  flUed   with  numerous  vessels;  the  nerve-cells   bave 

completely  disappeared. 
Z.  Latéral  column. 
P.  Posterior  column. 
T.  Tùrck's  fasciculus. 
Thèse  three  fasciculi  are  completely  healthy. 

PiG.  2. — Transverse  section  of  the  spinal  cord  in  the  lumbar  région. 
The  letters  bave  the  same  signification  as  in  Pigure  i. 
The  anterior  cornu,  C,  is  perfectly  normal,  and  contains  numerous  nerve- 
cells.     There  is  no  longer  any  sclerosis  of  the  anterior  radicular 
zone,  A. 

PiG,  3. — Middle  part  of  the  dorsal  région. 
A.  Anterior  radicular  zone,  sclerosed. 

C.  Anterior  cornu,  much  less  vascular  than  in  the  cervical  région,  and 
containing  one  or  two  nerve-cells. 

PiG.  4. — Différent  phases  of  the  destruction  of  nerve-cells. 
a,  h.  Cells  in  process  of  destruction. 
c.  Normal  cell. 

PiG.  5. — Longitudinal  section  of  the  phrenic  nerve. 

a,  a.  Normal  tubes,  in  which  the  myéline  is  coloured  black  by  osmium 
separated  from  each  other  by  broad  connective  bundles. 

PiG.  6. — Small  vessel  takenfrom  the  grey  substance  of  the  anterior  cornu, 
a.  Tumefied  cell. 

h.  Cells  containing  several  nuclei. 
c.  Nucleus  of  an  endothelial  cell. 

PiG.  7. — Vessel  takenfrom  the  same  région,  with  ils  walls  covered  by  numerous 
leucocytes. 


Voi.  11.  PI.  VII. 


.Fiq,  3, 


*>m>' 


Fia  .   5 


h 


Fi  o).  G. 


..^ 


g- 


WVst    N^W»i.LM    &   ' 


PLATE  VIII,   VOL.   II. 

PROTOPATHIC  MUSCULAK  ATROPHY. 

FiG.  I. — Transverse  section  of  the  phrenic  nerve. 

a,  a.  Section  of    fascicles,   where    the    retained    nerve-tubes   are   still 

rather  numerous. 

b.  Spaees  frora  which  the  nerve-tubes  hâve  totally  disappeared. 
(Drawing  taken  in  the  caméra  lucida.) 

FiG.  2. — Transverse  section  of  a  normal  phrenic  nerve.  (The  outlines  hâve 
been  drawn  in  the  caméra  lucida,  with  the  same  magnifying  power  as  in 
Fig.  I.) 

FiG.  3. — Tuheof  diseased phrenic  nerve  {parenchymatous  neuritis). 

a,  a.  Nuclei  contained  in  the  interior  of  Schwann's  sheath. 

b.  Fragmented    meduUary  matter.     The    axis-cylinder  has  disappeared. 

Magnified  about  700  diameters. 

Fig.  4. — Longitudinal  section  of  fibres  of  normal  diaphragm. 
Fig.  5. — Longitudinal  section  of  diseased  diaphragm. 

a.  Fibres  atrophied,  but  still  retaining  their  cross-striation.    They  are 

unequal  in  size.     The  connective  intervais,  b,  are  enlarged,  owing  to 

the  atrophy  of  the  muscular  fibres. 


Fi 


Vol.  ii.pi.  Yin. 


'.Wst  Neiyv.va»!  *  C?  .-A  Uth. 


PLATE  IX,   VOL.   IL 

LOCOMOTOR  ATAXIA. 

Spontaneous  fractures  of  left  radius  and  ulna. 


Vol.  II.  PI  K 


PLATE   X,   VOL   II. 

LOCOMOTOR  ATAXIA. 

Spontaneons  fractures  of  the  fémur.     Coxo-femoral  arthropathies  :  lésions  qf  the 
superior  extremities  of  hoth  fémurs. 

The  représentation  of  a  normal  fémur,  oij  the  left  of  the  plate,  is  given 
to  enable  an  exact  idea  of  the  lésions  to  be  at  once  obtained. 


Vol.  11.  PI.  X. 


..4  ; 


Jte.-«(| 


•V.^l 


West  Newm^n.  &  C  Uth.. 


// 


X 

x> 

O 
O 
-H 


/?J7 


W 


—I      "^1 


—  s  "3 


—1      —  a 


o 
c 
<•••) 

•y) 

«y) 
•H 

3 


••—1 


-j         — I 


— .  1 


21 


-1  c* 

1  n 

n  X 

o 

=1  o 


03 


19  D  en. 


Charcot,   J. 


Diseases  of  the  Nervous 
System  1881 


j 


#iiP 


y/,